Acute renal failure (ARF) is one of the renal expressions in patients with aplastic anemia (AA)-paroxysmal nocturnal hemoglobinuria (PNH) syndrome following hemolytic crisis. We report the case of an AA patient who experienced recurrent episodes of ARF, in association with evidence of PNH. A 46-year-old woman with AA was admitted because of oliguria and dark urine following a urinary tract infection (UTI) caused by Candida. PNH with ARF complication was diagnosed. Hemodialysis treatment was performed nine times and her renal function recovered. However, she suffered from recurrent hemoglobinuria and acute deterioration of renal function 4 months later. The renal biopsy showed features of acute tubular necrosis, deposition of hemosiderin and positive urate stain in the proximal tubular cells, without vascular thrombosis. The patient received management by adequate hydration, diuretics and alkalization. Her renal function recovered completely. This case report shows an AA patient experiencing recurrent episodes of hemoglobinuric ARF within a short period and achieving complete recovery of renal function after hemodialysis and suitable supportive treatment. In conclusion, AA might evolve into PNH and become complicated with presentation of severe ARF. It may occur with precipitating episodes, such as the UTI in this case. Early recognition of hemoglobinuric complications and prompt treatment for precipitating disease and ARF are important to prevent progression to an irreversible adverse renal outcome.