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Background and Objectives: Primary diaphragm tumors are rare. The aim of this study was to explore the clinicopathological features of primary diaphragm tumor patients who underwent surgical treatment in our center to improve the diagnosis and treatment of this disease. Methods: Clinical data of patients with primary diaphragm tumor who underwent surgery in our hospital from 2004 to 2019 were reviewed and analyzed. Results: A total of 18 patients were enrolled. The male:female ratio was 8:10, and the median age was 58 years old (35–74 years old). Most patients included in this study had no typical clinical symptoms. Nine tumor cases were distributed in the left and right diaphragms separately, whereas 11 cases were located at the diaphragm angle. The diaphragm of 12 patients was reconstructed by direct suture. All postoperative pathologies showed that the tumors were benign, and cysts were observed in most of the cases (5/18). Conclusions: There are no difference in distribution of gender and distribution on both sides of the diaphragm. In addition, primary diaphragm tumor is common in middle‐age patients. Most cases occur in the diaphragm angle and are characterized by cyst lesions. Surgical resection is an effective treatment option for primary diaphragm tumor. [ABSTRACT FROM AUTHOR]