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Ghrelin is a novel growth hormone-releasing peptide that causes a positive energy balance by stimulating food intake and inducing adiposity and has effects on growth. Many children with congenital heart disease (CHD) present with growth retardation and malnutrition owing to multifactorial reasons.To evaluate the circulating level of ghrelin in Egyptian children with congenital cyanotic and acyanotic heart disease and its relation to anthropometric measurements.The study included 40 patients with cyanotic and acyanotic CHD (18 cyanotic and 22 acyanotic) and 18 age- and sex-matched healthy control children. All children were subjected to measurement of height, weight, body mass index (BMI) and serum ghrelin was measured using ELISA technique.Weight, height and BMI were significantly lower in cyanotic and acyanotic patients compared to the control group (p=0.0001). Serum ghrelin levels were significantly higher in children with cyanotic and acyanotic CHD in comparison to the controls (p=0.0001). There was a significant negative correlation between ghrelin and BMI in the three groups (r=–0.534, p=0.023; r=–0.558, p=0.007; r=–0.608, p=0.007 respectively for cyanotic, acyanotic and the control groups).Circulating ghrelin level was elevated in children with congenital cyanotic and acyanotic heart disease, and was associated with a decrease in BMI. This elevation in ghrelin level may represent malnutrition and growth retardation in those patients as obvious by anthropometric measures too. This may suggest that ghrelin may have an important role as a compensatory mechanism in the regulation of the metabolic balance in them.