Abstract：Periosteal osteosarcoma (PO) is a rare, moderately malignant bone tumor that accounts for less than 2% of all osteosarcomas. Most clinicians lack experience in the diagnosis and treatment of PO. This article reports a case of removal and inactivation of tumor segments combined chemotherapy for tibial periosteal osteosarcoma, and combined with literature review, summarizing the epidemiological characteristics, morbidity characteristics, diagnosis and treatment experience of periosteal osteosarcoma, and making recommendations for the diagnosis and treatment of this disease.