We report a case of severe cholestasis and sicca syndrome after thiabendazole administration for Strongyloides stercoralis infection in a 26-year-old patient. Liver biopsy, performed 15 days after the onset of jaundice, revealed a marked paucity of bile ducts, and cholestasis rapidly progressed to biliary cirrhosis. Because of the progression of jaundice and the development of esophageal varices, orthotopic liver transplantation was performed, 18 months after the beginning of disease. The mechanism responsible for thiabendazole-induced biliary injury is unknown. The association between sicca syndrome and biliary disease suggests an immunoallergic mechanism against an antigen which could be common to the biliary, lacrimal and salivary duct epithelium.