A 37 year-old-woman was evaluated in 1993 for a chronic asymptomatic cholestasis. An endoscopic retrograde cholangiopancreatography showed the biliary tract compressed, and a mesenteric angiogram disclosed that the cause of biliary obstruction was a portal cavernoma. In addition, large esophageal varices with "red spots" were observed at endoscopy. Propranolol and ursodeoxicolic acid were started and the patient has remained asymptomatic to date. The biliary features of portal cavernoma are reviewed, as well as its pathogenesis, diagnosis and management. Portal cavernoma should be considered in the differential diagnosis of chronic cholestasis.