Primary pediatric osteosarcoma of the skull.

A 16-year-old adolescent boy presented with osteosarcoma of the left parieto-occipital bone with no history of radiation, Paget disease, or retinoblastoma. The tumor presented as a painless lump, which he attributed to minor trauma 5 months before presentation. Biopsy of the lesion was inconclusive. Complete surgical resection was attained. Pathology revealed a diagnosis of osteosarcoma, grade 1 to 2/3. Adjuvant chemotherapy commenced after tumor resection. Primary pediatric osteosarcoma of the skull is an exceedingly rare malignancy, with only a handful of cases ever reported. Complete surgical resection with negative margins is the key to optimizing disease-free survival. Adjuvant chemotherapy is recommended for high-grade tumors and in cases of incomplete resection.