Backgrounds and aims Sclerosing cholangitis (SC) is a chronic cholestatic liver disease characterised by inflammation and progressive bile duct fibrosis. Our purpose was to describe characteristics of SC in childhood.

Methods We performed a retrospective study of 3 childrens with SC followed in the paediatric department of Sfax (2008–2013).

Results There are 2 boys and 1 girl. The mean age at diagnosis was 6 years. Clinical features at presentation were jaundice (1 case) and hepatosplenomegaly (2 cases). Autoantibodies (anti-nuclear antibody, smooth muscle antibody, and perinuclear antineutrophil cytoplasmic antibodies) were detected in 1 case. Magnetic resonance cholangiography revealed irregularities with strictures, dilatations and pruning of bile ducts. Histological examination of liver biopsy showed signs of CS stage I (1 case), stage III (1 case) and portal inflammation with infiltration of lymphocytes and plasmocytes and periductal fibrosis (1case).

Search histiocytosis was negative in 3 cases. Colonoscopy with mucosal biopsies revealed no specific inflammatory colitis in one case. The diagnosis of overlap syndrome was made in one case and primary SC (2 cases). All patients were treated with ursodeoxycholic acid and the patient with overlap syndrome received immunosuppressive therapy. After a mean follow-up of 3 years, remission was noted in 2 cases and one patient progressed to cirrhosis and liver failure.

Conclusion SC is a rare cause of chronic cholestasis. Ursodesoxycholic acid is the treatment of choice for all forms of SC but without proof of its effectiveness in preventing progression to secondary biliary cirrhosis.