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Polyarteritis nodosa (PAN) is a rare multisystem vasculopathy that predominantly affects medium-sized arteries. Involvement of the cardiac and/or pulmonary vasculature may be fatal. We describe a 67-year-old Japanese male who presented with multiple coronary artery aneurysms and subsequently developed lower extremity gangrene. A diagnosis of PAN was established based on the correlation of clinical presentation and laboratory and imaging findings. In addition, we review other manifestations of PAN and differential considerations for this rare but potentially lethal condition.