Objective To investigate the pathological feathers,diagnosis,differential diagnosis and prognosis of esophageal granular cell tumor. Methods The data of seven patients of esophageal granular cell tumor diagnosed from 2008 to 2014 in our hospital were analyzed. Seven cases of esophageal granular cell tumor were studied by hematoxylin-eosin and EliVision immunohistochemical staining,the clinical and pathological feathers were analyzed with review of the literatures. Results The pathological feathers of seven cases were very typical. The tumors located in the submucosa,and showed gray to grayish yellow,nodular,with no capsule. The size of these tumors ranged from 0. 4 ~1. 0 cm in diameter. Microscopically,the tumor cells were round,polygonal or spindle with small central nuclei and rich eosinophilic granules cytoplasm,the nucleolus could not be easily seen. Immunohistochemical stain showed that the tumor cells were positive for S-100,CD68 and NSE,but negative for SMA,Desmin,CK,CD34,CD117,DOG1,HMB45. The proportion of Ki-67 positive cells was very low. Histochemical stain showed that the tumor cells were positive for PAS. All 7 cases were benign,and the follow-up time was from 8 months to 6 years without recurrence in patients. Conclusion Granular cell tumor of esophagus is very rare in soft tissue tumors,most of which are benign and located in the middle and lower segment of esophagus. The tumor has a better prognosis. The definite diagnosis should depend on the pathological di-agnosis.