Melanotic neuroectodermal tumour of infancy - A rare entity.

Melanotic neuroectodermal tumour of infancy (MNTI) is rare, rapidly growing, pigmented neoplasm of neural crest origin. It is generally accepted as a benign tumour despite of its rapid and locally destructive growth. It primarily affects the maxilla of infants during the first year of life. Surgical excision is considered as the treatment of choice. The recurrence rate varies between 10% and 15%, and malignant behaviour has been reported in 6.5% of cases. We report a case of MNTI, associated with an erupted primary tooth in a 5-month-old male child. We discuss the clinical, radiographic and histologic features of this rare tumour, as well as its surgical management and the follow-up.