A rare case of melanotic neuroectodermal tumor of infancy involving the right occipital bone in an 11-month-old infant is described. The bone tumor and its dural extension were surgically removed. Three-dimensional reconstruction of the tumor and brain from neuroimaging data added worthwhile information for preoperative planning. Microscopic examination revealed melanotic and neuroblastic tissue in a fibrillary matrix involving the expanded bone and superficial dura. A focal osteoblastic cranial mass in an infant should alert the clinician to consider this tumor.