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Objectives To present the clinical presentation, management, and pathological findings of a patient with osteosarcoma of the petrous apex with atypical metastasis to the lower abdominal wall. Methods Retrospective review of the records of a case of osteosarcoma of the petrous apex. Results A 49-year old man with facial pain is diagnosed with a right petrous apex lesion with biopsy demonstrating high-grade osteoblastoma. Multiple attempts at en bloc resection were not curative, therefore radiation and chemotherapy was recommended after two surgical attempts. The patient subsequently developed a cutaneous, lower abdominal wall mass which demonstrated osteosarcoma. Petrous apex tumor growth progressed despite treatment until the patient expired from burden of disease. Conclusions Temporal bone osteoblastoma and osteosarcoma are both extremely rare, and can be difficult to differentiate histologically. The present case illustrates these difficulties and demonstrates the possibility of malignant conversion from high-grade osteobastoma to osteosarcoma.

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