Introduction. Sclerosing cholangitis is characterized by an inflammatory and fibrotic lesion of intra- and/or extrahepatic bile ducts. When a causal mechanism of a bile duct lesion is identified, the sclerosing cholangitis is considered secondary. The vasculitis, including the Behçet disease, is cited as a probable cause of the ischemia and the sclerosing cholangitis. No cases of extrahepatic secondary sclerosing cholangitis have been reported to date. Case Report. We report the first case of secondary sclerosing cholangitis of the extrahepatic bile ducts associated with Behçet disease in a male who is aged 43, with a previous history of the angio-Behçet followed by complications of thrombophlebitis and a cerebral thrombophlebitis, and who has a cholestatic jaundice. The diagnosis has been carried out by the MR cholangiopancreatography which has objectified a moderate distension of the intrahepatic bile ducts upstream of regular stacked parietal thickening of the main bile duct. The patient has been treated successfully with the ursodeoxycholic acid and the placement of a plastic stent. Conclusion. This diagnosis should be mentioned to any patient with vasculitis and who has a cholestatic jaundice.