Extensive Melanotic Neuroectodermal Tumor of Infancy

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare childhood neoplasm with an alarming but classical clinical presentation. We present the case of a 2-month-old male infant treated with surgery for an aggressive MNTI on the alveolar process of the maxilla. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla, and displacement and dysmorphic changes in the developing primary tooth buds. The patient remained well without evidence of recurrence for 6 months after surgery. We discussed clinicopathological features, management alternatives, and outcome.