Melanotic neuroectodermal tumor of infancy is rare. Only 3 cases have been reported in the soft tissue of the extremities up to date. It has a typically biphasic feature in morphology. Epithelial and melanotic markers are positive in the epitheliod cells and neuron-specific enolase or synaptophysin is positive in the small blue round cells in immunohistochemistry. Radical resection and close follow-up is the treatment strategy in general situation. Here we report one case of MNTI in the upper extremity with review of the literature. This is the first case of MNTI in the forearm.
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