Four children, three males and one female, aged 1.5 months to 11 years, with restrictive cardiomyopathy are described. One had patent ductus arteriosus in association. Hepatomegaly, abnormal second heart sound and signs of pulmonary venous congestion in chest X-ray were the most consistent clinical findings. Diagnosis was based on echocardiographic findings, cardiac catheterization data and results of pathology. The most frequent echocardiographic sign was the enlargement of both atria but with the ventricles within normal size and normal systolic function. Abnormalities in second phase of the ventricular filling were recorded in 2D-echo Doppler and cardiac catheterization, where a dip and plateau morphology of ventricular tracings was recorded. Metabolic studies performed in two children were normal, and any child had hypereosinophilia. Two children died, one was lost for follow-up and another is asymptomatic. Remarks on medical and surgical management are made.