The melanotic neuroectodermal tumor of infancy (MNTI) is a rare benign neoplasm of neural crest origin most commonly found in the anterior region of the maxilla. The tumor almost always develops during the first year of life, although in some cases it can be present at birth. MNTI\'s present as a rapidly growing, painless expansile, partly pigmented mass. They are usually unencapsulated, with a tendency to occur as a single lesion. Local excision is the treatment of choice and is usually curative. Main sites for recurrences are the maxilla (57%) and the skull/brain (28.6%). Malignant transformation has been noted in approximately 6.5% of all cases and in 2% of maxillary tumors. The case of a 3-month old boy who presented with a loose primary maxillary left central incisor is discussed. The diagnostic and clinico-pathological features as well as tumor management and importance of a timely diagnosis are reviewed.
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