The melanotic neuroectodermal tumor of infancy is a rare benign neoplasm that appears as a rapidly enlarging mass, most commonly in the anterior part of the palate. The tumor is believed to be of neurocrestal origin. The case report of a 3-month-old infant with a rapidly growing melanotic neuroectodermal tumor of infancy is presented. The tumor was surgically excised, and no evidence of recurrence has been found.