Melanotic neuroectodermal tumor of infancy (MNTI) is a rarely occurring neoplasm of neural crest origin, appearing in children during the first year of life. MNTI is a benign tumor but is nonetheless locally aggressive; it usually originates from the maxilla and the mandible. Recognition of the diagnostic features of MNTI is important, so that it may be differentiated from benign lesions (dermoid or epidermoid cysts, vascular lesions) as well as from malignant lesions such as small round cell tumors. We present a case of a 2-month-old infant who developed right retroauricular swelling with extension through the calvarium and into the central nervous system. The tumor was totally resected. Aggressive benign or malignant lesions of the calvarium may entail dural infiltration and brain involvement, as in our case. Early diagnosis and surgery are thus mandatory for benign or malignant tumors of the calvarium.