We report a male patient suffering left ventricular noncompaction cardiomyopathy in combination with low-gradient aortic stenosis. Preoperative echocardiography and magnetic resonance imaging revealed the diagnosis. After aortic valve replacement, the clinical course was uneventful. Two years of follow-up confirmed that the general prognosis was primarily affected by impaired ventricular function, thrombembolism, and arrhythmias.