Introduction: Primary colorectal angiosarcoma is a highly rare malignant tumor. There is no standard treatment method for this disease. No treatment of rectal angiosarcoma with apatinib has been reported so far. Case Presentation: In the current study, an 87-year-old male presented with the symptoms of frequent defecation for more than one month in Hangzhou, China, in 2018. The patient was initially diagnosed with a rectal stromal tumor. The patient underwent ultrasound-guided transrectal mass puncture in the next treatment. However, immunohistochemical examinations confirmed the initial diagnosis of rectal angiosarcoma. The patient had advanced age and rectal angiosarcoma with metastasis; he had no surgical indications, and we tried to use apatinib 250 mg/d treatment to control the progression of the lesion. Then, he received apatinib, a novel tyrosine kinase inhibitor of vascular endothelial growth factor receptor-2 (VEGFR-2). The patient has been stable to apatinib with a dose of 250 mg daily by now. Conclusions: Apatinib may play an important role in the treatment of unresectable angiosarcoma.
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