Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis which is involved in renal and visceral arteries. PAN typically presents with systemic symptoms. However, spontaneous perirenal hemorrhage from renal vasculitic aneurysm is a rare complication. We report a case of fatal complication of PAN, which manifested by acute renal failure and spontaneous perirenal hemorrhage. A 72-year-old man was admitted to the hospital with complaint of dyspnea and generalized edema for 2 weeks. Physical examination revealed coarse and decreased breathing sound with moist rales on lung field and 3 (+) pretibial pitting edema. The laboratory findings showed a white blood cell count of 14,300/mm3, hemoglobin of 7.8 g/dL, blood urea nitrogen level of 83 mg/dL, creatinine level of 10.62 mg/dL. The electrolyte panel showed sodium 139 mEq/L, potassium 9.5 mEq/L, chloride 111 mEq/L. Therefore, we underwent emergency hemodialysis via femoral catherization by impression of acute kidney injury and uncontrollable potassium level. After emergency dialysis, his condition was slowly improved. On 15th hospital day, he complained sudden onset of right flank pain accompanied with hypotensive episode. An abdominal computed tomography showed massive perirenal hematoma around the right kidney. On renal angiography showed multiple aneurysmal dilatation of interlobar artery of the right kidney and we underwent coil embolization. We suspected the renal artery aneurysm due to vasculitis. However, the anti-neutrophil cytoplasmic antibodies and antinuclear antibody test were negative. Clinically, his vital sign was deteriorated and coagulation profiles were also prolonged. After 5 days of coil embolization, he died of disseminated intravascular coagulopathy due to massive perirenal hematoma. According to the radiologic findings, we diagnosed the polyarteritis nodosa by aneurysmal dilatation of interlobar arteries of right kidney. In conclusion, spontaneous perirenal hematoma is a serious and unusual complication of PAN, clinician should have high index of suspicion for preventing serious complication.