A 23-year-old female patient with factor VII (FVII) deficiency was admitted with severe hypertension and renal failure. Brachial arterial pressures were 230/120 and 220/115 mm/Hg on the right and left arms, respectively. There was no blood pressure difference between the arms. Renal artery Doppler ultrasonography revealed bilateral severe renal artery stenosis (RAS). Contrast-enhanced magnetic resonance imaging angiography (CE-MRA) revealed severe mural irregularities, contrast enhancement in the aorta and its branches, and long-segment stenosis starting in the abdominal aorta and extending into the proximal renal arteries. The diagnosis of Takayasu arteritis (TA) complicated by RAS in a patient with FVII deficiency was established. This is the first case of concomitant TA and factor VII deficiency in the literature. In conclusion, TA complicated with RAS should be kept in mind in the etiology of secondary hypertension, even when there is no blood pressure difference between the arms in patients. CE-MRA is an accurate, sensitive, and safe imaging method for diagnosing vasculitis, even in the early phases of the disease, and should be considered for evaluating the activity and response to treatment in patients with TA.