Abdominal sarcoidosis is an uncommon form of sarcoidosis. The clinical presentation of esophageal, gastric, small bowel, colon, appendicular, spleen, pancreas, and abdominal aortic sarcoidosis are discussed in this review. The differential diagnosis of abdominal sarcoidosis is extensive. Other granulomatous diseases including tuberculosis, fungal infections, parasitic diseases, inflammatory bowel disease, and Whipple’s disease should be excluded before making the diagnosis of gastrointestinal sarcoidosis. Corticosteroid therapy is the mainstay of medical therapy in abdominal sarcoidosis. Second line agents such as methotrexate are also discussed. Surgical intervention may be necessary in patients with bowel obstruction, perforation, or massive hemorrhage. The authors also provide their experience regarding preoperative pulmonary evaluation of patients with pulmonary sarcoidosis undergoing surgery.