Granular cell tumors (GCT) are uncommon neoplasms. There is controversy regarding the endoscopic diagnosis and treatment of esophageal GCT. We studied the endoscopic diagnosis and management of esophageal GCT among 23 patients identified in a single-institution pathology database. Medical records, pathology, and endoscopic images were reviewed. All patients underwent endoscopy and endoscopic ultrasonography (EUS), and endoscopic resection was performed in 10 patients. Seven of 23 patients had more than one esophageal GCT. Only six lesions exhibited a classic yellow discoloration. Among patients with a single GCT, three, four, and nine lesions were located in the proximal, middle, and distal esophagus, respectively. EUS showed hypoechoic, smooth-edged lesions usually confined to deep mucosa and submucosa. Standard forceps biopsy was diagnostic in 19 of 23 patients (83%). Ten GCT ≤ 10 mm in diameter underwent successful endoscopic mucosal resection without complication. The endoscopic appearance, location, and number of esophageal GCT are highly variable. Histological proof is still necessary for the differential diagnosis of this rare neoplasm. Endoscopic forceps biopsy is usually diagnostic. Endoscopic resection appears safe and effective in selected cases with lesions ≤ 10 mm.