The clinical characteristics and the microscopic and immunohistochemical characteristics of 1 case with alveolar soft part sarcoma (ASPS) were analyzed with a follow-up period and related literature was reviewed. The patient was admitted to the hospital with a right hip mass for 14 years. CT showed that an oval mass with slightly low density was in the right gluteus maximus. The mass was soft and the cut surface was solid. The tumor cells were large in size and had abundant cytoplasm, and nucleus was visible. The results of immunohistochemistry showed that tumor cells were positive for MyoD1, Myogenin, NSE, TFE3, and were negative for Desmin, CD31 and SMA with Ki-67 proliferation index less than 20%. But the vessel wall around the tumor cell clusters is positive for CD31 and SMA. PAS staining showed eosinophilic granules and crystals in the cytoplasm of tumor cells. This case expressed ASPL-TFE3 type 1 fusion gene mRNA by RT-PCR. ASPS mostly occurs in adolescents and young people, but most patients have early hematogenous metastasis, the final prognosis is poor. Combined with clinicopathological features and genetic diagnosis, we can make the correct pathological diagnosis of this disease. In this patient, multiple metastatic lesions of the lung and metastasis of the iliac bone occurred two years after surgical resection, and after the treatment with pazulopani (small molecule anti-angiogenic targeted drug), the tumor was significantly reduced. At present, the condition tends to be stable, but the clinical biological behavior of the tumor is difficult to determine and long-term follow-up of the patient is required.