The spectrum of inflammatory bowel diseases includes ulcerative colitis, Crohn’s disease, and unclassified inflammatory bowel disease. Ulcerative colitis is a chronic inflammatory disease of the lining mucosa of the rectum and colon. Crohn's disease is characterized by transmural inflammation that can affect any part of the lumen of the gastrointestinal tract from the oral cavity to the perianal area. Unclassified inflammatory bowel disease is a term used when the disease does not meet the usual criteria for ulcerative colitis or Crohn’s disease with repeated colonoscopies and biopsies. The pathogenesis of the disease is thought to involve complex interactions between genetics, risk factors, intestinal microbiota, and the immune response. Inflammatory bowel diseases are systemic diseases that are not limited to the gastrointestinal tract and may affect many organ systems. Extraintestinal manifestations (EIM) most commonly affect the musculoskeletal system, skin, hepatobiliary tract, eyes, mucous membranes, and less commonly the lungs, pancreas, kidneys, nervous and cardiovascular systems. 50% of patients develop at least one EIM during their lifetime, sometimes even before diagnosis. Some EIM are directly related to the activity of the underlying disease (arthritis, aphthous stomatitis, nodular erythema, and episcleritis), some have an independent course (ankylosing spondylitis, uveitis), and some may or may not be associated with disease activity (primary sclerosing cholangitis, gangrenous pyoderma). The symptoms of EIM can be very debilitating. EIM that are related to activity of disease, optimal therapy for IBD with careful monitoring of the patient is often sufficient to relieve symptoms. EIM that are independent of disease activity may require a multidisciplinary team and additional therapy.