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Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, benign neoplasm of neural crest origin that most commonly occurs in the first year of life. The craniofacial area is affected 90% of the time with the maxilla being the affected site in the majority of cases. It has a recurrence rate of up to 27% and is associated with malignant transformation in up to 6.5% of patients. The current case describes the clinical presentation and management of a 3-month old male infant with MNTI of the maxilla. Following initial presentation, the tumor grew rapidly to compromise the aerodigestive tract. Surgical resection was performed and the adjacent bone was burred. There has been no clinical recurrence over a 4-year period of follow up.