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Osteosarcoma of the skull has poor outcomes. This case report describes the presentation and clinical course of a patient who was diagnosed with osteosarcoma of the skull involving the cribriform plate. After her initial diagnosis, she developed esotropia with severe unremitting headaches. She received palliative radiation, followed by chemotherapy, and responded well. Her initial symptoms involving the cranial nerves subsided, and her response was sustained. This report illustrates the need to effectively treat osteosarcoma of the skul

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