Abstract
Melanotic neuroectodermal tumor of infancy is a rare benign but locally aggressive neoplasm of neural crest origin with a high recurrence rate. It usually affects infants of < 1 year of age. Involvement of maxilla of an infant is the most common presentation although cases outside this setting have been reported. We report the case of a 6-month-old male child presenting with intraoral swelling. Radiologically, a tumor was detected in the right maxilla, and wide local excision was done. Histological examination revealed typical biphasic population of cells with deposits of melanin pigment. Immunohistochemistry study was done confirming the diagnosis.