Differential diagnosis of the acute flank pain in a patient without nephrolithiasis is challenging in emergency department. Renal infarction should be suspected if accompanying cardiac arrhythmia, heart failure or valvular disease is present. Activated protein C resistance is the most common thrombophilia that triggers venous-obstructive conditions and rarely arterial thromboembolism. We described a young male with a history of non-ischemic dilated cardiomyopathy and coexisting activated protein C resistance presented with new onset acute flank pain due to acute renal infarction.