A 15-year-old girl with right tibial osteosarcoma, diagnosed 22 months previously, developed right orbital and skull base metastases, with symptoms including painful protrusion of the right eyeball and severe visual impairment. She underwent embolization of the metastatic tumor, local irradiation, and chemotherapy followed by intralesional resection of the mass because extensive involvement of the skull base precluded complete surgical resection. The best corrected visual acuity in her right eye initially decreased to 20/200, then dropped to no light perception after embolization, and then improved to counting fingers at 50 cm after radiotherapy. The symptoms of pain and proptosis subsided completely. After 24 months of follow-up from the presentation of orbital metastasis, the patient's right-eye vision remained unchanged. The surgical specimen revealed a necrotic tumor similar to osteosarcoma. In conclusion, orbital metastasis of osteosarcoma is rare, but may lead to severe visual impairment. Combined radiotherapy, chemotherapy and surgery can partially relieve symptoms, but cannot completely eradicate the tumors.