Portant un interet particulier pour la pathologie hepatique, et apres avoir collecte plus de 50’000 coupes histologiques, Gerald Klatskin decrit en 1965, pour la premiere fois, une famille particuliere de tumeurs malignes des voies biliaires extra hepatiques, situees au niveau de la bifurcation du hile hepatique. Cette entite est connue aujourd’hui dans la litterature sous le nom de «tumeur de Klatskin» et correspond a un adenocarcinome de l’epithelium biliaire. Il s’agit d’une tumeur rare, representant dans l’annee 2000 moins de 0.5% des nouveaux cas de cancer aux Etats-Unis. Les autres tumeurs malignes interessant la region peri-hilaire sont le cholangiocarcinome des voies biliaires intrahepatiques, l’adenocarcinome de la vesicule biliaire, ainsi que les tumeurs malignes primaires d’organes avoisinants, tels que carcinome hepatocellulaire et l’adenocarcinome de la tete du pancreas. On a rapporte egalement quelques rares cas de sarcome du hile hepatique (Soareset al., 1989), de neoplasies hematopoietiques (Eliason et Grosso, 2001) et occasionnellement, des metastases a distance d’un site primaire (i.e. cancers coliques, gastriques, pancreatiques, prostatiques, pulmonaires ou mammaires). Les tumeurs benignes comme le papillome (solitaire ou multiple, Fig. 1) ou le cystadenome, les pseudotumeurs inflammatoires (Sakaiet al., 2001), et les kystes ou pseudokystes de la voie biliaire principale (Seguchiet al., 2004) se presentent avec une Symptomatologie identique a celle observee dans les tumeurs malignes. Seules, les tumeurs du hile hepatique seront abordees dans cette revue. Les autres pathologies non-tumorales, telles que la cholangite sclerosante, la cholangite pyogenique recidivante, la lithiase biliaire (i.e. Syndrome de Mirizzi) ainsi que la stenose hilaire d’origine inflammatoire, traumatique ou iatrogene ne seront pas discutees ici. Il est toutefois important de savoir que 5 a 15% des patients operes avec un diagnostic preoperatoire de cancer du hile, presentent en effet une stenose inflammatoire pseudotumorale non-specifique (Santoroet al., 2004). Through his extensive interest in liver pathology, a collection of more than 50.000 histological slides, in concert with his keen interest in patients’ histories, Gerald Klatskin identified and first described in 1965 a distinct family of malignant tumours located in the bifurcation of extrahepatic bile ducts in the liver hilum (Klatskin, 1965). These tumors, which today carry his name «Klatskin tumours», are mostly adenocarcinomas typically originating from the bile duct epithelium. These tumours are rare, accounting for less than one half percent of all new cancers in the United States in 2000. Other malignant tumours that affect the perihilar region of the liver include cholangiocarcinomas of the intrahepatic ductal system, adenocarcinomas of the gallbladder, as well as primary malignant tumours originating from adjacent organs, such as hepatocellular carcinomas, carcinomas of the pancreatic head, rare sarcomas (Soares et al., 1989) and hematopoietic malignancies (Eliason and Grosso, 2001). Finally, occasional metastases from distant sites are found (i.e. from carcinomas of the colon, the stomach, the pancreas, the prostate, the lungs, or the breasts, to name only the most frequent primary sites). In addition to these malignant tumours, several benign tumours and tumourlike lesions cause similar symptoms, such as epithelial precursor lesions of the large extrahepatic bile ducts (i.e. solitary or multiple papillomas and/or adenomas, (Fig. 1)), rare inflammatory pseudotumours (Sakai et al., 2001), as well as occasional cysts or pseudocysts of the major duct systems (Seguchi et al., 2004). Not included in this review are nontumorous causes of obstructive jaundice such as primary sclerosing cholangitis, recurrent pyogenic cholangitis, stone disease (i.e. Mirizzi syndrome), as well as traumatic, inflammatory, or iatrogenic strictures. Indeed, these latter benign pseudotumoral strictures of mostly unspecific or unknown etiology are found in 5 to 15 percent of tumours surgically resected from the hepatic hilum for biliary stenosis (reviewed in: Santoro et al., 2004).