Melanotic neuroectodermal tumor of infancy is a very rare benign but locally aggressive neoplasm of neural crest origin with a high recurrence rate. It usually affects infants of less than 1 year of age. Most commonly, the lesion affects the maxilla followed by skull, mandible, brain and other extracranial sites. We report an 11-month-old female baby with a similar tumor involving the anterior region of maxilla along with a brief review of the literature and differential diagnosis. A wide surgical excision was performed by an intraoral approach. A 2-year follow-up of the patient showed no recurrence.