Introduction:

The melanoma neuroectodermal tumor of childhood (NMTCH) is a rare entity, usually benign, with rapid and expansive growth, with a high rate of recurrence. The objective is to present an unusual clinical case and review of the literature.

Clinical case:

Male patient, seven of months of age with a right retroauricular tumor, which has a progressive growth with a diameter of 5 x 3 cm, stony consistency, well-defined edges, adhered to deep planes. Surgical complete resection was achieved. The patient is free of tumor activity, after two-year of follow-up.

Conclusion:

The NMTCH is a neoplastic entity that must be considered as part of differential diagnosis of soft tissue tumors, particularly in head and neck, which can occur in both children and adolescents.