Background: Pediatric cardiomyopathy (CM) is a rare but serious and often life-threatening condition. In children, cardiomyopathy is often a part of multisystem disorder, which requires the attention of multiple subspecialists. Aim: The aim of this study was to estimate the prevalence of cardiomyopathy among infants and children with congenital heart disease (CHD) and to determine   the relationship of cardiomyopathy to type of CHD. Methods: This retrospective descriptive study was carried out in the Pediatric Cardiology outpatient clinic, Al Zahra Hospital , AssiutHospitalAl-AzharUniversity, during the period from January 2012 to September 2014, to review the files of patients diagnosed with cardiomyopathy. The study included 60 cases included 29 (48.3 %) cases with DCM, 28 (46.7%) cases with HCM and 3 (5%) cases with RCM The age of studied cases ranged from 10 days to 8 years with mean age 20.61± 25.62 month .The files of all of these patients were reviewed for the following data: file number, name, age, sex, address, date of diagnosis, frequency of follow up, date of last follow up, presenting symptoms, clinical manifestation, consanguinity, other affected sibling, previous viral infection, history of drug intake and  investigation done including electrocardiogram, chest X-ray and echocardiographic data. Results: The most common clinical presentation was dyspnea (86.6%) followed by respiratory infection (48.3%), followed by palpitation (15%). According to different types of congenital heart diseases, our study showed that patent foramen oval (PFO), patent ductus arteriosus (PDA) and atrial septal defect (ASD) were the main congenital heart defects in the isolated shunt lesion where they represented 50%, 25% and 22.7% respectively. Consanguinity was positive in 50% of patients, while a family history of another affected sibling was positive in (10%). Conclusion: The most prevalent form of CHD among cardiomyopathic patient was the isolated shunt lesion followed by combined obstructive and shunt lesion then isolated obstructive lesions and lastly the cyanotic lesion. Our results reveals highly significant difference between types of cardiomyopathy and left ventricular end diastolic diameter (LVEDD), left ventricular end systolic diameter (LVESD) .