A case of melanotic neuroectodermal tumor of infancy (MNTI) in a 6-months-old white female child is presented. The tumor was located in the left half of the superior maxillary bone and affected the maxillary sinus, inducing large facial asymmetry. The histologic aspects of the tumor were typical for the entity: two cell populations, one pigmented melanocyte-like cell, the other small neuroblastic-like cell.
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