Objective: To investigate the clinicopathological features of oral melanotic neuroectodermal tumor of infancy (MNTI). Methods: We retrospectively reviewed the clinical and pathological data of 13 cases of oral MNTI treated in Peking University School and Hospital of Stomatology from 1980 to 2007. Results: The age of patients ranged from 2 to 7 months old. Nine lesions occurred in the maxilla, 3 in the mandible and 1 in the cheek. The most common symptom of the disease was swelling. Five tumors were found purple-reddish or blue-blackish. The neoplasm grew rapidly. Only 1 case was diagnosed as MNTI and 3 cases were diagnosed as malignant tumor before biopsy. Two of 9 cases with follow-up information recurred in one month after surgery and 1 case dead of the tumor. One case with remnants of tumor did not recur after 19 years postoperatively. Conclusion: Oral MNTIs appear to occur in certain locations and people of certain age. Special attention should be paid to the color of the tumor during intra-oral examination. This tumor grew rapidly and invasively and the patients require close follow-up within the first 6 months postoperatively. The pathologic differential diagnosis of MNTI should include other pe-diatric "small round cell" neoplasms. Conservative excision is the preferred treatment choice.