Primary osteosarcoma of the skull (POS) in a young man with intracrani
al involvement is reported. After an initial transient remission by su
rgical intervention and chemotherapy, he began to deteriorate due to t
umor recurrence and intracranial hemorrhage, and died 15 months follow
ing the time of diagnosis. The rarity and poor prognosis of POS are em
phasized together with the review of the clinical and therapeutic aspe
cts in the previously reported 98 cases in the literature.