Primary sclerosing cholangitis is an uncommon disorder of obliterative biliary inflammation that is exceedingly rare in childhood. Presented is a child with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia) and primary sclerosing cholangitis. This case should focus attention on the association of primary sclerosing cholangitis and immunodeficiency syndromes, particularly in children with immune disorders and unexplained hepatic abnormalities.