A 1-year-old female infant presented with a large 7-8 cm Ewing's sarco
ma of the left parietal calvarium. It deeply indented the subjacent he
misphere. Wide surgical excision was followed by chemotherapy but not
radiation. The patient was clinically free of tumor when she expired 9
months later of sepsis. Though primary Ewing's sarcoma of the skull i
s said to be relatively rare, we have tabulated 37 cases of this disor
der, including the present one (we excluded mandibular tumors (>100 ca
ses) since they are not usually treated by neurosurgeons). The mean ag
e was ii years and of those available to follow-up, three quarters wer
e tumor-free 6 months to 7 years postsurgery. The remainder survived a
n average of 21 months. Ewing's tumors of the calvarium generally gran
t a favorable prognosis following surgery and/or radiation and chemoth
erapy. Those at the base of the brain may be more difficult to eradica
te. In addition to primary Ewing's tumors, metastases to the skull and
the blain from other primary sources in the skeleton have been docume
nted in several reports.