We describe a 66-year-old man who developed renal failure related to granulomatous renal sarcoidosis without systemic manifestations. Renal failure was severe enough to require hemodialysis transiently. Renal biopsy of this patient revealed the central necrosis of the granuloma which is usually absent in sarcoid granuloma. Serum level of angiotensin converting enzyme (ACE) was not helpful for diagnosis in this patient because serum ACE level is often elevated in the condition of chronic renal failure. Immunohistochemical detection of ACE was of diagnostic value in this patient. Subsequent course in which glucocorticoid was used for therapy was consistent with the diagnosis. This is the first report of identification of ACE in renal sarcoid granuloma.