A 17-year-old boy with Duchenne's muscular dystrophy and congestive cardiomyopathy with a left ventricular thrombus is described. The patient presented with flank pain, and computed tomography of the abdomen revealed multiple bilateral renal infarcts. An echocardiogram delineated a left ventricular thrombus and generalized hypokinesis with a left ventricular ejection fraction of 25%. Heparin therapy was started, but the patient died of refractory congestive heart failure. Autopsy revealed diffuse skeletal myopathy consistent with Duchenne's muscular dystrophy as well as biventricular cardiomyopathy with a recent left ventricular apical-septal mural thrombus. Right atrial thrombus, a left upper lobe pulmonary embolus, and splenic and renal infarcts were also noted. To our knowledge, this is the first reported case of left ventricular thrombus with or without systemic emboli in the cardiomyopathy of Duchenne's muscular dystrophy.