Extraintestinal and systemic manifestations occur commonly in patients with inflammatory bowel disease, specifically ulcerative colitis and Crohn's disease, and affect most all organ systems of the body. The occurrence of such widespread manifestations strongly suggests that these disorders are systemic in nature and may have a common mechanism. Extraintestinal manifestations may be incidental findings that cause no symptoms, but more commonly complicate the management of the underlying inflammatory bowel disease, being a source of considerable morbidity and mortality. Some extraintestinal manifestations not only correlate with a specific disease state but also with the location, extent, and degree of activity and disease. Most extraintestinal manifestations found in patients with inflammatory bowel disease involving the small intestine appear to correlate with some underlying pathophysiologic mechanism.