Melanotic Neuroectodermal Tumour of Infancy (MNTI) is an uncommon neoplasm found predominantly in infants, with around 220 cases reported so far in world literature. 92% of the reported cases have been found in children less than one year of age and 82% of cases have been within the first 6 months of infancy [1, 2, 3]. This tumour has a predilection for the craniofacial region and is most frequently found in the maxilla (72%) followed by cranial vault (12%), mandible (5%) and brain (4%) [4, 5]. The remainder of the sites where the lesion has been reported are epididymis, mediastinum, ovaries and few isolated cases in the thigh, foot, scapula, cheek and zygoma. The earlier uncertainty on the origin of the tumour resulted in a variety of terms [6, 7] for the lesion. Krompecher (1918) who first reported this neoplasm referred to it as congenital melanocarcinoma. The other names proposed have been melanotic progonoma, pigmented ameloblastoma, retinal anlage tumour, melanotic epithelial odontoma, and pigmented congenital epulis [8]. Borello and Gorlin in 1966 referred to the tumour as melanotic neuroectodermal tumour of infancy and suggested neural crest origin on the basis of high excretion of vanillyl mandelic acid [9] by the patients which is characteristic of tumours of neural crest origin, namely pheochromocytoma, neuroblastoma and ganglioneuroblastoma [10, 11]. The neural crest origin has further been established from the support received on the basis of immunohistochemical, ultrastructural and biochemical analysis. MNTI is a locally aggressive benign lesion with a recurrence rate of 15%. About 16 cases of malignant variants (6-7%) reported, have been from CNS. Keeping in mind the locally aggressive behaviour of this neoplasm, its tendency for recurrence and the highly proliferative nature of its constituent cells, the management proposed for this lesion is early surgery in the form of either enucleation with aggressive curettage or enbloc resection, depending upon the extent, proximity of the lesion to vital anatomic structures and the histopathological report.