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Myocardial noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy characterized by excessively protrusive trabeculae and deep trabecular recesses in one or more segments of the ventricle. A 37-year-old male patient with prominent kyphoscoliosis presented with dyspnea. Transthoracic echocardiography showed trabeculations and deep intratrabecular recesses along the ventricular cavity in the apical and lateral segments of the left ventricle. Color Doppler imaging showed blood flow in these recesses. Diameters of the left ventricle and atrium were increased, with global hypokinesis of the left ventricle (ejection fraction 30%). Severe aortic valve stenosis was noted. Pulmonary function test showed moderate restriction. Due to high perioperative mortality, the patient was scheduled to medical treatment.