The granular cell tumor is a solitary painless nodule that arises most commonly on the skin or the tongue. The vast majority are benign. Approximately 5% to 9% of granular cell tumors have been reported in the gastrointestinal tract, most commonly in the esophagus. We report a case of a 45-year-old African American woman with multifocal granular cell tumors of the esophagus and proximal stomach. Two lesions within the distal esophagus and proximal stomach were characteristic nodular granular cell tumors. Within the mid esophagus there was poorly defined transmural involvement by benign-appearing granular cells. This pattern of infiltration by benign cells is uncharacteristic. A review of the literature with emphasis on the determination of malignancy is also presented.