RESUMEN
Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, fast-growing, pigmented neoplasm of neural crest origin. Despite of its rapid and locally infiltrative growth, it is still considered benign with high recurrence rate and malignant potential, so early diagnosis is extremely important to limit its local expansion. Recurrences can be expected primarily because of incomplete excision, tumor dissemination, or due to its multicentric nature. We report a case of MNTI originating in the maxilla of a 7-month-old male infant, which was managed with complete surgical excision of the tumor, with no signs of recurrence after a 1-year follow-up period. In addition, a complete literature review has been reported, in an attempt to understand the origin, histopathologic and immunohistochemistry features with surgical modalities and advantages of radiotherapy and chemotherapy in cases where complete surgical extirpation is questionable.