Extraintestinal complications of patients with ulcerative colitis contribute to quality of life and mortality of the patients. Extraintestinal complications involve many organ systems. Skin, mucocutaneous, hepatobiliary, and joints complications are major extraintestinal complications. Some disorders correspond the activity of the colitis, other complications develop independently of the bowel inflammation. The purpose of this review is to update the clinicians concerning the etiology, clinical course and the therapy of the extraintestinal manifestations associated with ulcerative colitis.