Benign recurrent intrahepatic cholestasis is a rare disorder of unknown etiology and has not yet been reported in Taiwan. We report a case with a typical clinical course. A 17-year-old Taiwanese boy had three episodes of pruritus and jaundice from February 1993 to July 1995, each lasting 3 to 4 months. Jaundice spontaneously subsided and he was symptom-free during periods of remission. A fourth episode of pruritus began in July 1995, with jaundice developing later and lasting for 3 months. Laboratory tests revealed direct hyperbilirubinemia. Endoscopic retrograde cholangiopancreatography showed normal intra- and extrahepatic biliary trees. Light microscopy of a liver biopsy sample revealed hepatocellular and canalicular cholestasis with bile retention in the Kupffer cells. Benign recurrent intrahepatic cholestasis was diagnosed after exclusion of other possible causes of jaundice. The patient made an uneventful recovery.