Sarcoidosis is a multi-system disorder characterized by non-caseating epithelioid granulomas in multiple organs. The disease usually presents in young adults and is uncommon in children. Renal involvement can usually occur due to granulomatous interstitial nephritis, but renal failure is uncommon. Corticosteroids are the mainstay of therapy. We present the report of a child with severe renal failure secondary to renal limited sarcoidosis who was successfully treated with corticosteroid induction therapy. Because of the severe side effects of corticosteroids, mycophenolate mofetil was added and corticosteroids were tapered off. The child has been in sustained remission for over a year with mycophenolate mofetil monotherapy.