Idiopathic isolated right ventricular cardiomyopathy is an extremely rare cause of right ventricular failure. Here, we report a case of 65-year-old male presented with chief complaints of dyspnea, fatigue, and bilateral pedal edema for the last 6 months. On clinical evaluation, grade II/III pansystolic murmur was present in left parasternal area. Investigations and imaging revealed dilated right atrium and ventricle with normal pulmonary artery pressure without any etiology. Magnetic resonance imaging ruled out other common causes of right ventricular cardiomyopathy; thus, the patient was diagnosed as a case of idiopathic isolated right ventricular cardiomyopathy that is a diagnosis of exclusion.