Melanotic neuroectodermal tumour of infancy.

Melanotic neuroectodermal tumour of infancy is an extremely rare neoplasm arising in newborns and young children, typically involving the face or cranium. A case arising from the maxilla, requiring extensive resection with a near-total maxillectomy, is presented. A thorough review of the literature on this unusual tumour is provided, with emphasis on prognostic factors and appropriate treatment.