Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. The origin of the tumor is the neural crest. The expansive, destructive, and rapid growth of MNTI and its effects on the surrounding tissues are the most obvious clinical features. Microscopically, large polygonal epithelioid cells resembling melanocytes, with variable deposits of melanin, and smaller neuroblast-like round cells characterize MNTI. Malignant transformation may occur. Since the first description in 1918, only 215 cases were reported up to the last extensive review in 1992. The present review supplements another 140 published cases of MNTI up to 2004, including an original case report. Clinical features, treatment alternatives, and follow-up are discussed.