Abstract Background Aortoiliac occlusion disease, also called Leriche syndrome, is characterized by atherothrombotic obliteration of the aortic bifurcation and bilateral common iliac arteries; typically, it has a chronic presentation. Pulmonary embolism is more related to venous thromboembolism rather than arterial thromboembolic events. Therefore, cases of simultaneous acute Leriche syndrome and pulmonary embolism are rare. Existing intracardiac right-to-left shunt were detected in most previous cases. Herein, we present the first likely documented case wherein acute Leriche syndrome and pulmonary embolism occurred simultaneously without a patent foramen ovale. Case presentation A 58-year-old man with hyperlipidemia and coronary artery disease presented with a 4-h history of bilateral lower limb numbness. He was a heavy smoker with a history of stroke. Computed tomography angiography revealed pulmonary embolism and aortoiliac artery occlusion. Although a massive thrombus straddled the bilateral pulmonary arteries, orthopnea was his only presentation, without right ventricle failure. Cyanosis of the affected limbs was noted, and muscle strength in both limbs had regressed to grade 1. Owing to acute limb ischemia, he underwent an emergency operation to salvage the limbs. On postoperative day 5, the general condition of both the legs improved; the muscle strength improved to grade 4. He was then transferred to the general ward and enoxaparin was continued. Computed tomography angiography was repeated to evaluate the pulmonary embolism on postoperative day 8; the thrombus remained lodged in the bilateral main pulmonary arteries. Owing to persistent orthopnea and chest tightness with intermittent tachycardia, he underwent a staged operation for the pulmonary embolism on postoperative day 13. During the surgery, intraoperative transesophageal echocardiography showed no patent foramen ovale or an existing right-to-left shunt. Postoperatively, he was closely monitored in the intensive care unit for 3 days and then transferred to the general ward for 10 days. A final computed tomography angiography performed on postoperative day 18 revealed thrombus resolution. He was then discharged on postoperative day 30 without any in-hospital complications. Conclusion We present a case that might be the first documented report of acute Leriche syndrome co-occurring with pulmonary embolism without an existing patent foramen ovale.