OBJECTIVE AND IMPORTANCE:Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally aggressive tumor that arises most commonly from the maxilla or mandible. Infrequently, it originates from the cranial vault, and recent reports have described a favorable outcome after radical surgery. Some lesions are particularly problematic, such as those located along the cranial midline or cranial base and those with significant intracranial extension. Currently, there is no effective adjuvant therapy for MNTI; radiation is precluded by the patients’ young age, and chemotherapy trials have not demonstrated long-term efficacy.CLINICAL PRESENTATION:A 2-month-old infant boy presented with a firm, immobile subcutaneous mass behind the right ear. The mass had been present at birth and enlarged with time.INTERVENTION:Initial resective surgery down to the dura resulted in massive tumor recurrence within weeks. Successful management required repeat surgery including excision of the dura and dural venous sinuses.CONCLUSION:This patient’s large MNTI of the cranial base was successfully managed by radical surgery. Although MNTI is a rapidly growing tumor that is locally highly invasive, radical surgery may be associated with a favorable outcome and offers the potential for long-term cure.