Melanotic neuroectodermal tumor of infancy is uncommon and has rapid expansile growth. Melanotic neuroectodermal tumor of infancy is a very rare and benign but locally aggressive neoplasm that originates from neural crest and is composed of relatively primitive pigment-producing cells. The lesion commonly affects the maxilla of infants during the first year of life. Common treatment methods include surgical excision and resection of the tumor. The aim of this article is to present the diagnosis and treatment of a patient with melanotic neuroectodermal tumor that occurred in the anterior maxilla of a 6-month-old male baby.