Renal infarction is a condition induced by renal artery occlusion and causes renal parenchymal damage. The most common cause of renal infarction is atheroembolic cardiac diseases. Other causes are trauma, autoimmune diseases and hypercoagulability. Homocysteine is a vasculotoxic substance, and mutations in the Methyltetrahyrofolate Reductase (MTHFR) enzyme may result in an increase in serum homocysteine levels. In this report, we present a 35-year-old case with bilateral renal infarction, for which we hold the MTHFR C677T mutation and hyperhomocysteinemia responsible, because of its rarity.