Extraintestinal manifestations of ulcerative colitis and Crohn's disease are symptoms of varied organ injuries due to systemic inflammatory process. Bowel disease seems to be primary but upon genetic disposition disturbed immune regulation may result in inflammation of other organs that may appear simultaneously, precede bowel disease or follow it in the quiescent phase. As far as possible extraintestinal manifestations have to be distinguished from the complications of the intestinal inflammation and also from side effects of drugs for its treatment. Involvement of bones and joints, skin, eyes, liver and biliary tract is more common but disease of blood coagulation system, lung, kidneys and nervous system may also appear. Treatment of extraintestinal manifestations is influenced by their association with the activity of the bowel disease. Beyond some specific interventions, to achieve improvement of manifestations independent from activity long lasting anti-inflammatory therapy or immunomodulation is almost always needed. Clinical evidence of different levels from reports support the favorable effect of biologic therapy in different extraintestinal manifestations of inflammatory bowel diseases as well. Care and follow-up of patients is a multidisciplinary task.