Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease strongly associated, in the western series, to inflammatory bowel disease (IBD) and particularly to chronic ulcerative colitis. North African data are rare. aim : To study the epidemiological, clinicobiological and morphological profile of PSC in Tunisia. methods: A retrospective multicenter study extended over a period of 14 years (1995-2009), including all patients suffering from PSC, hospitalized in the four participants departments. We collected epidemiological, clinico-biological, histopathologic and morphological data for each patient. results: We brought together 33 patients (22 men and 11 women), middle aged 44 years. The disease was symptomatic in 73% of cases. Laboratory tests showed cholestasis (100%) associated with hyperbilirubinemia (72%) and a moderate cytolysis (78%). Morphological analysis of bile ducts by cholangioMRI or endoscopic retrograde cholangio-pancreatography objectified diffuse damage of the biliary tract in 61% of cases. Association with IBD was found in 33% of cases (Crohn's Disease: 27%, chronic ulcerative colitis: 6%). An association with autoimmune hepatitis was found in 6% of cases and primary biliary cirrhosis in 3% of cases. Conclusion : PSC is rare in Tunisia and affects men more often than women. The association with IBD is less frequent than in literature. It concerns essentially Crohn's disease. These data require confirmation by prospective multicenter studies.