We reported 5 cases of granular cell tumors (GCTs) of esophagus and reviewed the literature. There were 4 females and 1 male with a median age of 43 years and an average age of 44 years. All of the cases had solitary tumors. Tumor size was 0.4-2.5 cm in diameter. Gastroscopy revealed that 2 cases were located in the middle esophagus, 1 case in the upper esophagus, and 2 cases in the distal one. Five cases displayed gray-white, pink, yellow mucosal uplifts of esophagus, 3 cases had smooth surface, 1 case was slightly concave, and the biggest tumor had erosion. Tumor cells were large and polygonal with rich granular and eosinophilic cytoplasm, and small oval nuclei. Cells were arranged in nest or aciniform. Immunohistochemistry and histochemistry staining showed S-100+, neuron specific enolase+, Vim+, CD68+, smooth muscle actin-, Des-, CK-, CD117-, CD34-, Ki67-or ≤ 5%+. Periodic acid-Schiff reaction and epithelial membrane antigen were both weakly positive. GCTs of esophagus are rare and most of the cases have good prognosis.