Pulmonary hypoplasia or aplasia is part of the spectrum of malformations characterized by incomplete development of lung tissue. In more than 50% of these cases, coexisting cardiac, gastrointestinal, genitourinary, and skeletal malformations are present, as well as the variations in bronchopulmonary vasculature. In literature there is no reported case describing lung hypoplasia with dilated cardiomyopathy without structural heart disease. Here, it is presented a 6-month girl, referred to us for persistent homogenous opacity in left hemithorax detected on chest X-ray. Subsequently, she was found to have dilated cardiomyopathy without structural congenital heart disease and congenital hypoplasia of left lung.