In 1987, a patient presented with pulmonary sarcoidosis and progressive renal failure. Percutaneous renal biopsy showed focal glomerular sclerosis (FGS). Over the subsequent 4-year period, her renal failure progressed to require hemodialysis. During this time, her pulmonary sarcoidosis was treated with daily corticosteroid therapy and she remained in clinical remission. The patient received a one-haplotype-identical living-related renal transplant from her mother. The patient is now 25 months posttransplant with a serum creatinine of 106 mumol/L (1.2 mg/dL). Her sarcoidosis continues to be in clinical remission. In this report, we review the rare association of a primary glomerular lesion causing renal failure in patients with sarcoidosis. In addition, we detail the first successful renal transplant in such a patient.