Introduction: Granular cell tumors are rare, usually benign, neoplasms presenting as solitary small nodules in the skin or subcutaneous tissue. Involvement of the gastrointestinal tract is unusual, particularly of the appendix, and it is characterized by indolent, submucosal lesions usually diagnosed as an incidental finding.
Case report: We describe the rare case of acute appendicitis secondary to a granular cell tumor of the appendix in a 19-year-old male.