Objective: To investigate the clinicopathologic features and differential diagnosis of low grade appendiceal mucinous neoplasm (LAMN). Methods: We retrospectively analyzed clinical details, histopathological features and follow-up data of 5 cases diagnosed with LAMN. Results: Clinical symptoms of patients diagnosed with LAMN consist of right lower abdominal pain and discomfort; right lower quadrant abdominal mass, sometimes accompanied by peritoneal pseudomucinous tumor with specific pathological and cytological diagnostic characteristics. Conclusion: LAMN is a rare disease and easy to be misdiagnosed. Accurate pathological diagnosis is essential for optimal management of patients with LAMN.