A 67-year-old woman was referred to our hospital due to a refractory lower extremity ulcer. Occlusion of the bilateral superficial femoral arteries and a difference (>50 mmHg) in blood pressure between the bilateral upper limbs were noted. In addition to occlusion of the left subclavian artery and stenosis at the ostium of the right coronary artery, these findings led to a diagnosis of Takayasu arteritis. Furthermore, a biopsy of the ulcerated skin lesion localized on the fibular surface showed a non-caseating cutaneous granulomatous lesion resulting in the diagnosis of cutaneous sarcoidosis. The simultaneous occurrence of cutaneous sarcoidosis and Takayasu arteritis, albeit rare, should not be overlooked.