Inflammatory bowel disease (IBD) is associated with extra-intestinal manifestations (EIMs) that tend to parallel intestinal activity and have a debilitating effect on the quality of life. EIMs primarily affect the joints, skin, and eyes with less frequent involvement of the liver, kidney, and pancreas. This article reviews the prevalence of musculoskeletal, dermatological, ocular, and other manifestations in IBD and their coalition with underlying intestinal inflammation. EIMs occurring independently of intestinal activity are managed by targeted therapies, categorical regimens, and specific treatments. On the other hand, EIMs paralleling the bowel activity are carefully monitored while the IBD is brought under control. Since the etiology of the disease is responsible for the development of the EIMs, the research scrutinizes the identified pathogenic mechanisms that tend to involve genetic susceptibility, aberrant self-recognition, and autoantibodies directed against organ-specific antigens shared by intestinal and extra-intestinal organs. This article also provides an overview of the epidemiology, clinical features, diagnostic modalities, and management of the EIMs associated with IBD.