Takayasu's arteritis is a chronic inflammatory arteriopathy of unknown cause. The pulseless phase of Takayasu's arteritis is preceded by a period of "prepulseless disease" when the patients suffer from systematic symptoms without obvious evidence of obstruction of larger arteries. Herein we report a unique case of Takayasu's arteritis presenting as sarcoidosis manifested by restrictive lung disease, hilar adenopathy and non-caseating granulomas of the skin. We are aware of only one report in which non-caseating skin granulomas were evident at the prepulseless phase of Takayasu's arteritis. However, in our patient the clinical picture was typical of sarcoidosis. Increased awareness of this possibility will lead to more frequent skin biopsies in patients with Takayasu's arteritis and nodular skin lesions, which may increase the number of reported cases with this combination.