Antimyocardial autoantibodies are a cause of dilated cardiomyopathy (DCM). Immunoabsorption therapy for eliminating autoantibodies can improve cardiac function in adult DCM. The purpose of this study was to investigate the indication and efficacy of plasma exchange in children with DCM and their outcomes. We performed a single-center, retrospective study in children with DCM who had received plasma exchange (PE). Six patients in various degrees of heart failure (three patients in acute exacerbation phase, one patient in early phase, and two patients in chronic phase) received PE. The effects of first PE were that the left ventricular ejection fraction (LVEF) and New York Heart Association (NYHA) functional class were transiently increased in five of six patients (83 %) and in four of five patients (80 %), respectively. The median duration of improved cardiac function after first PE was 8 months. PE was performed a total of two times in two patients and three times in one patient. The effect of repeated PE was attenuated when compared with first PE. Improved LVEF and NYHA functional class were observed in two of four courses (50 %) and in one of four courses (25 %), respectively. The median duration of improved cardiac function was 1 month. PE can transiently improve cardiac function and clinical symptoms of DCM in children. PE may be an additional therapeutic option in children with refractory DCM. However, PE should only be considered as a bridge to ventricular assist device implantation or heart transplantation.