A 2-month-old boy underwent surgery for removal of a right temporal melanotic neuroectodermal tumor of infancy (MNTI). Histologically the tumor tissue showed signs of malignancy. The child was reexamined several times up to the age of 5 years. Neuroradiological evaluation showed no evidence of tumor recurrence or metastases. No resulting handicap was observed during neurological and psychological follow-up examination at the age of 5 years. Our findings confirm that surgical removal as the therapy of choice provides an excellent prognosis for this kind of tumor in spite of its histologically malignant appearance.