Objectives: To identify malnutrition in children with Down Syndrome (DS) and congenital heart disease (CHD). To examine the long-term effects of correction intervention. Methods: Data (n=181) were obtained from the health records (from 1984 to 2007) of all children (0-172 months) with DS followed at the Santa Casa de São Paulo Hospital, Brazil. Cases with mosaicism and /or coexistent major pathology were excluded. Detailed anthropometry was performed and z-scores calculated. Malnutrition was defined as weight height z-score < 2, 0. Patients with CHD admitted for corrective intervention were evaluated for nutritional status before and after surgery (6 months; 1, 2, and 5 years). Results: 165 children with DS were analysed,the others were excluded. 67, 8% (n=112) was a sample of children with CHD and 32, 1%(n=53) without heart disease.58 children were admitted for corrective intervention. Mean age of correction was 25, 4 months (+/- 35,495). Before the surgery the mean weight z-score was -2, 6448 (+/- 1, 68) and mean height z-score was -3,037 (+/- 1,862). The time elapsed to weight height was on average one to two years after surgery (Table I/ groupI= age of correction until 84,05 months and Group II after this age ). The patients with greater growth disturbance present better recovery on postoperative growth (Tables I and II/ Groups I, II and IIII= age of correction until 56,03, between 56,04 and 112,07 months after this age). Conclusions: Malnutrition is common in children with CHD and SD. Corrective intervention results in significant improvement nutritional status.