Objective:To investigate the clinical features, diagnosis, treatment and prognosis of gastrointestinal neurilemmoma.Methods:A descriptive study was conducted to retrospectively analyze the clinical manifestations, endoscopic findings, pathological features, treatment methods and prognosis of patients with gastrointestinal neurilemmima diagnosed from May 2017 to November 2020 in the Second Affiliated Hospital of Xi'an Jiaotong UniversityResults:A total of 19 patients were collected, ranging in age from 45.0 to 70.0 years old, of whom 68.4% (13/19) were female. The clinical symptoms are non-specific, and some patients are asymptomatic (15.8%(3/19)) and may present with abdominal pain (57.9%(11/19)), nausea (31.6%(6/19)), abdominal distension (21.1%(4/19)) and decreased appetite (21.1%(4/19)), and a few with gastrointestinal bleeding (5.3%(1/19)). Tumors were most frequently found in the gastric body (42.1%(8/19)), followed by the duodenum (15.8%(3/19)). Endoscopic submucosal mass is easy to be misdiagnosed as stromal tumor. The pathological type showed shuttle cell tumor, immunohistochemical staining showed positive S100 and Vim, most Ki-67 staining proliferation rate was <5% (57.9%(11/19)), and a small part was ≥10% (10.5%(2/19)). Treatment was either endoscopic or surgical. No tendency to recur and metastasize was found in any of the neurilemmimas.Conclusion:Gastrointestinal neurilemmoma is a very rare submucosal tumor of the digestive tract, most of which are benign and rare in malignancy, and postoperative pathology is the gold standard for its diagnosis. Gastrointestinal neurilemmoma is common in middle-aged female, with diverse and non-specific clinical symptoms, difficult to differentiate from submucosal tumors such as stromal tumors, and its tendency to malignant transformation increases with tumor enlargement. Endoscopic resection is recommended for tumors with tumor diameter <3 cm without serosal invasion, with little trauma and no recurrence.