Ulcerative colitis is a chronic relapsing inflammatory disorder of the colonic mucosa of unknown etiology. The inflammatory process involves the mucosa and submucosa in a continuous segment of bowel with rectal involvement in almost all cases. Since its etiology is unknown, therapy is directed at modulating the inflammatory response in order to control symptoms and to prevent relapses. 5-aminosalicylates and corticosteroids have been the most widely used therapeutic agents for treatment of ulcerative colitis. Recently, experience has been gained with the use of other immunomodulators, such as mercaptopurine, azathioprine, methotrexate, cyclosporine, and tacrolimus, in pediatric patients. Colectomy is indicated in patients with severe colitis who do not respond to intensive medical therapy. The care of children with ulcerative colitis not only involves control of symptoms from gastrointestinal and extraintestinal manifestations, but also optimizing growth and development. The complications of chronic inflammation and long-term medical therapy must be weighed against the risks and benefits of surgery for children and adolescents with this condition.