A 50-year-old man complained of dyspnea on exertion. Electrocardiography showed frequent premature ventricular contraction and inverted T wave in leads V1-V6. Echocardiography and right ventriculography showed dilation and systolic dysfunction of the right ventricle. The diagnosis was arrhythmogenic right ventricular cardiomyopathy. In addition, thrombus was also observed in the pulmonary artery on chest computed tomography and pulmonary artery angiography. The final diagnosis was arrhythmogenic right ventricular cardiomyopathy complicated by pulmonary embolism. Pulmonary embolism should be considered in patients with arrhythmogenic right ventricular cardiomyopathy with dyspnea.