Propionic acidemia is a rare autosomal recessive inborn error of metabolism. It is relatively common in Middle East. Dilated cardiomyopathy is one of the leading causes of morbidity and mortality for patients with propionic acidemia. Liver transplantation has been used for patient with frequent metabolic decompensations and was shown to be beneficial in propionic acidemia-related dilated cardiomyopathy. Up to our knowledge, there has been one reported case of recurrent dilated cardiomyopathy 3 years after liver transplantation. We report the first case, from Middle East, of recurrent dilated cardiomyopathy, 6 years after liver transplantation.