Background: The lipid profile is an important and independent predictor of coronary heart disease is. This process relates to cholesterol concentration, accumulation and deposition of lipids on the arterial wall. Objective: The aim of this study was the measurement of serum lipid profile (cholesterol, triglycerides, high-density lipoprotein (HDL) and low-density lipoprotein (LDL) in children with congenital heart diseases and to compare the results with those of healthy children. Patients and methods: This case-control study was carried out from July 2020 to December 2020 in Cardiology Unit of Pediatric Department Outpatient Clinic, Zagazig University Hospital on 44 children. Patients were divided into: Group (I): (Case group) included 22 children with congenital heart diseases, and Group (II) (control group) included 22 healthy children. All patients were subjected to dimensional transthoracic echocardiography with color flow Doppler (Vivid 7 dimension apparatus) and Laboratory investigations (total cholesterol, triglycerides, lowdensity lipoprotein (LDL), high-density lipoprotein (HDL). Results: The most frequent cardiac defect was ASD (22.7%) followed by VSD (13.6%), common A-V canal (9.1%), mitral regurgitation (9.1%), pulmonary regurgitation (9.1%), and PDA (9.1%). 18.2% of the studied patients had cyanotic congenital heart disease (CHD) and 81.8% had non-cyanotic CHD. There was statistically significant differences between the studied groups in lipid profile as case group had higher cholesterol, triglyceride, LDL and lower HDL than control group. Conclusion: We concluded in this study that patients had significantly higher serum lipid levels than age and sex-matched controls in light of these findings.