Melanotic neuroectodermal tumour of infancy arising in the maxilla

Melanotic neuroectodermal tumour of infancy is an uncommon neoplasm that usually occurs in children aged one year or less. Difficulty in deciding the cellular origin of this tumour has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumour. Electron microscopy and histochemical studies, however, have now established the neural crest origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. We present two cases of melanotic neuroectodermal tumour of infancy arising in the maxilla.