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, low-density 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.