A 42-year-old woman with a longstanding history of systemic lupus erythematosus and insulin-dependent diabetes mellitus was admitted to the hospital because of icterus, anorexia, pruritus, and weight loss of 3 months duration. During her evaluation the diagnosis of primary sclerosing cholangitis was established. The following case documents the unusual association of systemic lupus erythematosus and insulin-dependent mellitus with primary sclerosing cholangitis.