Background and Objective: Children with Down syndrome (DS) are prone to feeding difficulties, congenital heart defects (CHDs) and defective energy intake though they are often overweight when compared to contemporaries. This study was designed to evaluate the nutritional status of DS children with and without associated CHD. Materials and Methods: This study included 80 patients with DS recruited from the Genetic and Pediatric Cardiology Clinics at the Children's Hospital, Ain Shams University. Patients were classified into two groups according to the presence (group I) or absence (group II) of CHD. Dietetic history was taken using a 24 h dietary recall and food frequency questionnaire. Data were analyzed into macro- and micro-nutrients and referred to as a percentage from the recommended daily allowance (RDA). Anthropometric measurements were interpreted using Z scores. Results: Malnutrition, stunting and wasting were detected in 23.8, 45 and 11.3% of patients, respectively. Their prevalence rates were significantly higher among the group I (34.2, 55.3 and 21.1%) when compared to group II (14.3, 35.7 and 2.4%), respectively (p<0.05). Group I had significantly lower mean values of daily intake of energy, carbohydrates, fat, proteins, iron, calcium, sodium, potassium, phosphorous, selenium, vitamin B1 and vitamin D when compared to group II (p<0.05). Conclusion: Children with DS associated with CHD are more prone to nutritional complications. Nutritional rehabilitation strategy, including multiple micronutrient supplementations, is crucial in the management of those children with early involvement of dietitians and caregivers.