Extraintestinal complications affect 25-30% of patients with ulcerative colitis. These extraintestinal disorders significantly contribute to morbidity and mortality of ulcerative colitis patients. While some disorders parallel the activity of the colitis, other abnormalities run a clinical course independent of the bowel disease. The pathogenesis of these disorders is unknown, but the variable relationships to the severity of colitis and the variable responses to a proctocolectomy suggest considerable heterogeneity. The present therapy for the various manifestations is reviewed in depth. In this respect it is important to note that colectomy should never be mandated by the extraintestinal complications of ulcerative colitis.