This report describes an exceptional case of popliteal artery thrombosis secondary to exostosis of the superior extremity of the superior tibia in a young adult. Correct diagnosis was made during re-operation for recurrent thrombosis. Surgical treatment consisted of resection of the bony tumor and venous bypass to reestablish arterial continuity. Femoropopliteal vascular complications of exostosis are rare, with most cases involving arterial aneurysms or false aneurysms. Differential diagnosis in our young patient took into account the other causes of popliteal thrombosis: entrapped popliteal artery, adventitious cyst, fibrodysplasia, and juvenile arteriopathy. In patients with major functional disability, operative treatment is recommended to remove the bony abnormality and repair the arterial lesion.