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 200 instances of this tumor are reported in the medical literature. We present a case of newborn with MNTI on the left maxillary alveolar ridge. The patient underwent complete surgical resection without maxillectomy. There has been no recurrence in a follow-up period of 1 year. The diagnostic features and management alternatives of MNTI are discussed. (c) 2005 Elsevier Ireland Ltd. All rights reserved.