Ulcerative colitis and Crohn's disease have reported associations with a spectrum of extraintestinal diseases. This association has been appropriately termed as extraintestinal manifestations because large case studies have demonstrated that between 25% to 36% of patients with either type of inflammatory bowel disease will have at least one such associated disease. The spectrum of diseases reported associated with either ulcerative colitis or Crohn's disease involves many organ systems. Some of these extraintestinal manifestations are related to active colitis--joint, skin, ocular, and oral; small bowel dysfunction--cholelithiasis and nephrolithiasis; and nonspecific manifestations such as amyloidosis and hepatobiliary diseases. The purpose of this review is to update the clinicians with the most recent literature concerning the etiology, clinical course and the therapy of the extraintestinal manifestations associated with both forms of idiopathic inflammatory bowel disease.