Granular cell tumor (GCT) is an uncommon esophageal neoplasm. It commonly presents as a nonspecific painless mass. The purpose of this study is to describe our experience with 5 patients newly diagnosed with GCT at VA Medical Center in Memphis, Tennessee from February 2001 to June 2005. Clinical manifestation, endoscopic appearance, histology, different modalities of treatment and prognosis are discussed. Based on this experience, we conclude that GCTs are relatively rare in occurrence. They usually present as a firm to hard submucosal nodule on esophagogastroduodenoscopy. Diagnosis can be made by endoscopic biopsy in most cases. Treatment options include endoscopic surveillance, endoscopic resection, or surgery. The usual course of GCTs is benign.