We describe the case of a 47-year-old man who developed significant acute, and subsequently chronic, kidney injury due to bilateral renal infarction. This occurred in the context of a combined inherited thrombophilia including antithrombin III deficiency and a prothrombin gene mutation. Bilateral renal artery thrombosis developed despite prophylactic treatment for thromboembolism. Arterial thrombosis is rare in the context of inherited thrombophilia and bilateral renal infarction is an unusual cause of acute kidney injury. Bilateral renal infarction due to primary renal artery thrombosis has not been previously described in antithrombin III deficiency, either as an isolated defect or in combination with other hereditary thrombophilia.