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A granular cell tumor (GCT) is an unusual benign soft tissue tumor that can occur at any age and in any part of the body. GCTs are mostly found in the skin and subcutaneous tissues, bronchi, esophagus, breast tissue, and tongue. A GCT originating in the digestive tract, particularly in the appendix, is relatively rare and usually diagnosed as an incidental finding. We herein describe the first case of abdominal distension and occasional pain secondary to a GCT of the appendix in our hospital. The findings from this case suggest that a GCT of the appendix is a rare entity for which surgical resection is an efficient therapy.