Melanotic neuroectodermal tumour of infancy (MNTI), first reported by Krompecher in 1918, is an uncommon pigmented tumour affecting predominantly the craniofacial bones of the newborn infants 1. Although classically benign, it is rapidly growing, locally aggressive and can follow a malignant course2. Infants in the first year of life are usually affected, compelling prompt diagnosis and treatment as well as close monitoring 3. According to Krompecher, this tumor derives from epithelial nests evolved at the time of embryonic fusion of the facial processes. It has also been suggested that the tumor arises from the retinal anlage by a pinching-off process of neuroepithelium during the formation of embryonic eye7. Recurrences can be expected primarily because of incomplete excision, tumor dissemination during surgery, or multicentric nature . Approximately, a few hundred of these tumors have been reported in medical literature. We describe the case of a 3-month-old boy who presented with an enlarging swelling of left maxillary alveolus since 1 month of birth.
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