AbstractBackground and Aim: Hepatocellular carcinoma (HCC) is usually associated with chronic liver diseases and liver cirrhosis, while conversely, cholangiocarcinoma (CC) usually occurs in a non‐cirrhotic liver. The purpose of the present study was to evaluate CC in liver cirrhosis.Methods: Between January 1998 and December 1999, 26 patients with CC were retrospectively reviewed. The occurrence of CC in chronic hepatitis B infection‐related liver cirrhosis, portal vein thrombosis (PVT) and survival were analyzed.Results: Twenty‐six patients with CC (19 with a non‐cirrhotic liver and seven with chronic hepatitis B infection‐related liver cirrhosis) were included in the present study. All cases of CC in the cirrhotic group were incidentally discovered during routine screening for HCC. The mean age (± SD) was 58.8 ± 14 years in the cirrhotic group and 73.2 ± 15.9 years (P = 0.001) in the non‐cirrhotic group. When compared to the cirrhotic group, the non‐cirrhotic group had a higher median level of albumin (42 compared to 30 g/L, P = 0.005), bilirubin (117.5 compared to 18 µmol/L, P = 0.01), alkaline phosphatase (291.5 compared to 100 U/L, P = 0.001) and gamma glutamyl transpeptidases (215.5 compared to 31 U/L, P = 0.001). In contrast, the cirrhotic group had a higher median prothrombin time (PT) compared to the non‐cirrhotic group (18.2 compared to 12 s, P = 0.05). In the non‐cirrhotic group, only one patient (5.3%) showed evidence of PVT on a computerized tomography and Doppler ultrasound, while in the cirrhotic group six patients (85.7%) had PVT (P < 0.001). The median survival period in the cirrhotic group was six months (range 2–24 months) compared to 16 months (range 6–41 months) in the non‐cirrhotic group (P = 0.036).Conclusion: CC in cirrhotic liver presented at a younger age and patients who developed CC were prone to PVT. The survival period was also shorter in comparison to that of non‐cirrhotic liver patients.