Congenital dyslipidaemias are generally grouped into disordersleading to hypercholesterolaemia, hypertriglyceridaemia and mixedhyperlipidemia.Dyslipidemia can alter vascular endothelial function and impair some of its pro-fibrinolytic and anti-thrombotic regulatoryproperties, as well as initiate the atherosclerotic process. There are strong links between vascular changes and hyperlipidemia in children. This study summarizes an approach to the diagnosis and treatment of seventeen patients and their families through complete history taking, clinical examination and lipid profile for all patients before and after treatment with special emphasis on thyroid profile, fasting blood sugar, liver enzyme, urine analysis and creatinine to exclude secondary forms of dyslipidemia.Familial hypercholestelemia (FH) was the most common type. Both sexes were affected, positive family history was reported in fifty percent of total cases, positive consanguinity was reported in seventy five percent of total cases, and fifteen percent of total cases were discovered accidentally during family screening. Milky plasma and acute pancreatitis were more prevalent in familial hypertriglyceredemia (FHTG), while cardiac complicationswere more prevalent in familial hypercholestelamia. The patients offamilial hypercholesterolemia had better response to treatment than the other types of congenital dyslipidemias. Conclusion: The rate of lipid testing among children was low so morbidities associated withdyslipidemia were common. Children should be screened for dyslipidemia if there is positive family history of premature ischemic heart disease and/or dyslipidemia. Positive consanguinity is more prevalent in our country so congenital dyslipidemia should be taken more in consideration.