The association between autoimmune pancreatitis and sclerosing cholangitis has attracted considerable attention. In contrast to type 1 (IgG4-related) autoimmune pancreatitis, bile duct involvement is uncommon in type 2 autoimmune pancreatitis, a more benign condition characterized histologically by granulocytic epithelial lesions (GELs). Following our recent report on a child with GEL-positive sclerosing cholangitis and excellent response to steroids, we retrospectively reviewed the liver histology of a large number of patients with sclerosing cholangitis to investigate the possible role of type 2 autoimmune pancreatitis in this pathology. Liver biopsies of 103 children with autoimmune sclerosing cholangitis and 142 adults with primary sclerosing cholangitis were reviewed for the presence of neutrophilic bile duct injury. Histologic findings were correlated with clinical features, response to treatment, and outcome. Neutrophilic bile duct lesions similar to GEL were identified in 5 cases (4 children and 1 adult; 4% of autoimmune sclerosing cholangitis and 0.7% of primary sclerosing cholangitis). GEL was more commonly seen in wedge biopsy specimens. One patient had concomitant pancreatitis. Cholangiograms showed diffuse stricturing of bile ducts in all cases. The number of liver tissue IgG4 plasma cells did not increase, and serum IgG4 levels were normal in 3 patients tested. All patients went into remission with prednisolone and/or ursodeoxycholic acid, and their liver function tests remained completely normal without relapses over a follow-up period of 6 to 16 years. Although rare, the diagnosis of sclerosing cholangitis with GEL is important in view of its excellent and apparently sustained response to immunosuppressive treatment.