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Melanotic neuroectodermal tumor of infancy is a rare congenital neoplasm involving the head and neck region in young patients. A case of melanotic neuroectodermal tumor of infancy is presented. This tumor occurred in right maxillary alveolar ridge in a 4-month-old infant. The present case showed an increased urinary level of vanilmandelic acid, confirming that the tumor is originated from neural crest. Clinical assessment, histologic diagnosis, and laboratory findings supported the diagnosis.

Melanotic neuroectodermal tumor of infancy: Report of a case associated with high urinary excretion of Vanilmandelic acid