The clinical data of 6 acute promyelocytic leukemia (APL) patients with thrombosis as the first manifestation were retrospectively analyzed. Among 6 patients, 5 were males and 1 female.The median age at diagnosis was 55 years old. All patients had risk factors for cardiovascular disease (CVD), and 5 patients met the diagnostic criteria for disseminated intravascular coagulation (DIC). There were 3 patients at low risk (bcr1 subtype), 1 at intermediate risk (bcr2 subtype) and 2 at high risk (1 bcr3 subtype and 1 unknown). FLT3-ITD mutations were tested in 3 cases, all of whom showed negative results. Arterial thrombosis was found in all 6 cases, 4 cases had cerebral infarction, 1 had lower limb arterial embolism, and 1 had multiple arterial and venous thrombosis. Four patients with cerebral infarction received all-trans retinoic acid (ATRA) combined with arsenic trioxide (ATO)±chemotherapy and symptomatic treatment (1 patient at high risk did not receive ATRA), 2 patients achieved complete remission (CR), and the other 2 patients died of cerebral hemorrhage and cerebral infarction, respectively. One patient with lower extremity arterial thrombosis died suddenly within 12 h after admission likely due to acute myocardial infarction. One patient with mixed thrombosis received low molecular weight heparin and rivaroxaban successively after inferior vena cava filter implantation, and achieved CR after ATRA+ATO treatment. Thrombosis is a less common and under-recognized presentation in APL.Thrombosis patients with blood cells and/or coagulation abnormalities should consider the possibility of APL. APL patients complicated with thrombosis have a high probability of DIC and remain mostly intractable to existing treatments, who are at high risk of death and poor prognosis.