Objective To know the disease better by investigating the clinical features,analyzing characteristics of the cases and summarizing the experience of Multiple endocrine neoplasia type-1 (MEN-1). Methods Collect the clinical data of 10 patients with MEN-1 in the First Affiliated Hospital with Nanjing Medical University from January 2010 to June 2022,including demographic data, laboratory tests, tumor components and treatment methods and so on. The data of 24 cases of MEN-1 reported from January 2010 to June 2022 are retrospectively analyzed by searching PubMed database. Results A total of 34 MEN-1 patients are enrolled in this study, including 21 males and 13 females, with the onset median age of 55 years (30-80 years old). Among them, 20 patients have family medical history and 10 patients have metastasis. The most common tumor components are parathyroid adenoma (29 cases, 29%) , pancreatic neuroendocrine tumor (30 cases, 30%), pituitary adenoma (20 cases, 20%). 29 cases of parathyroid adenoma were treated surgically.28 cases of pancreatic neuroendocrine tumor were also treated surgically , including 2 cases of postoperative chemotherapy, 2 cases of postoperative biological agents for patients, and other 2 cases of long-term proton pump inhibitor therapy. Among 20 cases of pituitary adenoma, 5 cases were treated surgically, 8 patients were treated with drugs and 7 patients were followed up. Up to June 2022, only 1 patient died among 34 patients with MEN-1. Conclusion The clinical manifestations of MEN-1 are diverse. Parathyroid tumor, pancreatic neuroendocrine tumor and pituitary tumor are the three most common clinical manifestations of MEN-1. Surgical treatment is the main treatment methods of each tumor, and other therapies include biological agents, chemotherapy and so on. Key words Multiple endocrine neoplasia type 1（MEN-1），clinical characteristics，treatment