Abstract: Background: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm of neural crest origin, primarily containing melanin, and affecting the maxilla of infants during the first year of life. The tumor is benign but may grow very rapidly and invade the bone marrow. Very rare examples of malignant transformation have also been reported. Treatment consists of surgical removal, somewhat more aggressive than the typical “conservative” removal.
Case Report: A 4-month-old male was admitted to our clinic after the parents noted a rapidly growing swelling in the anterior maxilla 3 weeks previously and difficulty in oral feeding and weight loss due to malnutrition.
Conclusions: This tumor is an uncommon benign tumor of neural crest origin occurring in infants. The lesion exhibits a good response to surgical excision, with few recurrences, although long-term follow-up is advised because of the possibility, albeit rare, of malignant change.

Keywords: Maxillary neoplasm, infant, melanotic neuroectodermal tumor, infant oral health