Melanotic neuroectodermal tumor of infancy (MNTI), an uncommon neoplasm that involves predominantly the maxilla, is classically benign but clinically aggressive. Surgery is the mainstay of the treatment with a proven efficacy, but its optimal method is not entirely clear. A case of maxillary MNTI in a 4-month-old male infant treated by radical surgery without recurrence after more than 3 years of follow-up is presented. On the basis of this experience and a review of the literature, the optimal method of MNTI surgery is discussed. To improve the prognostic of this neoplasm, the role of conservative surgery should be limited to the treatment of tumors where clear surgical margins excision should require sacrifice of functional or vital structures.