Inflammatory bowel diseases (IBD) are systemic disease that manifest not only in the gut/GI tract but also in many patients in extraintestinal organs. The quality of life of IBD patients may be significantly affected by those extraintestinal manifestations (EIMs). It is important to have knowledge of the prevalence, pathophysiology and clinical presentation of EIMs in order to adapt therapeutic options to cover all aspects of IBD. EIMs may occur in up to 24% of IBD patients before the onset of intestinal symptoms and need to be recognized to initiate appropriate diagnostic procedures.