Melanotic neuroectodermal tumour of infancy (progonoma)

A case of Melanotic neuroectodermal tumour of infancy (melanotic progonoma, melanoameloblastoma, retinal anlarge tumour) presenting as tumour of maxilla in a female infant is reported. Clinically a teratoma was suspected but ultrasound shoved it a solid mass. At exploration, characteristic melanotic pigmentation clinched the diagnosis which was later confirmed by histopathological examination.