Melanotic neuro-ectodermal tumor of infancy is very rare. A unique neoplasm of the formative years, it typically involves the face or cranium; possesses rapid, expansile growth; presents as a firm swelling and displays a high rate of recurrence. Its rarity and unique features make diagnosis and management quite challenging. This challenge is increased in resource-limited settings like ours and with atypical presentation, such as was seen at our center, where patient presented with a bony hard swelling.
Keywords: Melanotic neuro-ectodermal tumor of infancy; benign tumor; bony-hard swelling; diagnostic challenges; firm swelling; infant; management challenges; maxillary tumor; vanyl mandellic acid; wide excision.