Gastrointestinal stromal tumor(GIST)have an insidious onset,and most patients have distant metastases at the time of diagnosis.The common metastatic sites are liver and peritoneum,while bone and lung metastases are rare.A patient with multiple metastasis of GIST complicated with main pulmonary artery embolism was admitted to the First People's Hospital of Chenzhou in April 2022.The patient was hospitalized due to cough and chest tightness.The patient was found to have main pulmonary artery embolism and a huge pelvic mass by assisted examination,and the puncture biopsy of abdominal mass revealed spindle cell tumor.Immunohistochemistry showed CD117(+),CD34(+)and Dog-1(+).The patient was treated with tyrosine kinase inhibitor(TKIs)imatinib mesylate(IM),and patient's condition was stable after anti-coagulation therapy with low molecular weight heparin and warfarin.Through this case introduction and literature review,it is concluded that pulmonary embolism is rare in GIST patients.For these patients,no matter the embolism is tumor embolism or thrombus,anticoagulation therapy can reduce tumor load and relieve embolism.In clinical practice,compre-hensive modes including surgery,intervention,chemical therapy and targeted therapy are usually adopted.It is expected to provide reference for future treatment of GIST to strive for better clinical prognosis.