Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, fast-growing, pigmented neoplasm of neural crest origin. It primarily affects the maxilla of the infants during the first year of life. Approximately, a few hundred of these tumors have been reported in medical literature. We present a case of a newborn with MNTI involving the anterior maxillary region. The treatment included surgical excision of the lesion with safe margins, using an intraoral approach and removal of associated developing tooth buds. We made no attempt at immediate bone grafting. The patient had no recurrence at 1 year postoperatively. The diagnostic features and management alternatives of MNTI are discussed.