Abstract
Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon pigmented neural crest tumor primarily found in infants. We presented a case report of successful surgical management of MNTI in an 11-month-old female. A total of 178 articles discussing 249 cases of MNTI were identified through literature search. Literature review of 250 cases of MNTI including the current case report was conducted considering study parameters such as age and gender of the patient, location of the lesion, levels of vanillylmandelic acid, management options, and outcome after treatment. Statistical review of the data showed that MNTI predominantly affects the anterior maxilla in infants less than six months of age. Recurrence of the lesion shows a significant association with age of the individual and treatment method employed. This study reports a 2.4% mortality rate, 2% malignancy rate, and a recurrence rate of 15.2%, with recurrence times ranging from 15 days to 20 months. We advocate a minimum follow-up of four months to two years to monitor recurrence.