Melanotic neuroectodermal tumor of infancy (MNTI) is a rare pigmented neoplasm of neural crest origin that most commonly arises in the maxilla of neonates and infants during the first year of life. We report the case of a three-month-old female child who presented with a 15-day history of intraoral swelling. Radiologically, the tumor was detected in the right maxilla, which was removed by wide local excision. Histopathological examination revealed the biphasic population of cells with melanin pigment deposition. Immunohistochemistry study was done confirming the diagnosis of MNTI.
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