BACKGROUND Esophageal granular cell tumor (eGCT) is a relatively rare, usually benign neoplasm of the neuroectoderm. It is derived from Schwann cells. Clinical symptoms of this disease are non-specific. However, the most common presenting symptom is dysphagia, which is mostly misdiagnosed as esophageal polyps under gastroscopy, yet it has a 2% chance of forming cancers. We report the case of a 52-year-old woman with solitary eGCT, then analysed retrospectivelyanalyze the clinical features and elucidate on the reduction of misdiagnosis and missed diagnosis. CASE SUMMARY A 52-year-old woman was diagnosed with "esophageal granulossoma" after esophagoscopy, which was mistaken for eGCT. CONCLUSION eGCT diagnosis depends on characteristic pathomorphologies and detection of the S-100 protein. Endoscopic mucosal resection is the preferred therapeutic method.