A 37-year-old female patient was admitted with exertional dyspnea. Her serum creatinine was 2.4 mg/dL and anti-nuclear antibody was positive in a titer of 1/320. Renal biopsy revealed diffuse proliferative lupus nephritis. Echocardiography and cardiac magnetic resonance (MR) imaging showed increased apical trabeculations compatible with left ventricular noncompaction (LVNC), which is a rare genetic cardiomyopathy. The patient expressed a marked improvement in exertional dyspnea after the immune-suppressive treatment for systemic lupus erythematosus (SLE). Control echocardiography revealed a significant increase of ejection fraction. SLE may cause a kind of cardiomyopathy with high resemblance to LVNC.Discrimination of these two similar clinical entities is important because SLE-induced cardiomyopathy is potentially reversible after the immune-suppressive treatment for SLE.