Gastrointestinal (GI) tract sarcoidosis is an uncommon form of sarcoidosis. The GI tract can be involved as an isolated disease as a part of systemic sarcoidosis. Clinical manifestations of esophageal, gastric, small bowel, colon, and appendicular sarcoidosis are discussed in this review. The differential diagnosis of GI sarcoidosis is extensive. Other granulomatous diseases of the GI tract, like tuberculosis, fungal infections, parasitic diseases, inflammatory bowel disease, and Whipple's disease, should be excluded before making the diagnosis of GI sarcoidosis. Corticosteroid therapy is effective in treatment of GI sarcoidosis. Surgical intervention may be necessary in patients with bowel obstruction, perforation, or massive hemorrhage.