A 71-year-old male with hypertrophic osteoarthropathy (HOA) was referred to our hospital because of a nocturnal fever and tenderness stretching from the left parotid region to the left front neck, in which antibacterials were ineffective. He was diagnosed with Takayasu’s arteritis (TAK) following findings of contrast-enhanced computed tomography and neck ultrasound. This is the first report to describe the development of TAK after a prolonged course of typical HOA and proposed HOA and TAK have common underlying pathophysiological factors.