Melanocytic neuroectodermal tumor of infancy: excision and primary palatal repair at 7 months of age.

Melanocytic neuroectodermal tumor of infancy is a rare pigmented tumor generally occurring in the head and neck region in children 12 months of age or younger. The true incidence is unknown and fewer than 200 clinical case reports have been reported to date in the literature. The tumor itself is locally aggressive. It does not have a tendency to metastasize but carries a high risk of local recurrence. This report describes one patient's care, including primary palate reconstruction, and provides a review of the literature. A 7-month-old boy was found in a Haitian orphanage with a growing, very large mass originating from his right maxilla.