Introduction: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally invasive, fast-growing neoplasm with a high recurrence rate, which usually affecting children under 1 year of age. The tumor originated from the neural crest, most commonly found in the head and neck region, especially in the maxilla. Current standard for treatment is by surgical excision, but the high recurrency rate (10% - 60%) and the possiblity of malignant transformation makes the extent of excision debatable. Case report: An 8 -months-old boy was referred to our department with a rapidly growing mass in the anterior of the maxilla since two months prior. The mass was bluish in color, firm, and non-ulcerative. We treated the patient with excision under general anesthesia. Pathology examination was done and MNTI was confirmed. There is no recurrency at 2 years follow-up. Summary: Diagnosis and treatment of MNTI must be done meticulously due to the aggressive nature of the tumor. Wide surgical excision is the best treatment for this case, and routine follow-up must be done to detect any reccurency. Keywords: melanotic neuroectodermal tumor; infancy; maxilla, surgical excision