Background and Objectives:Children with congenital heart disease (CHD) are prone to malnutrition. This can have a significant effect on the outcome of surgery. Our objective was to determine the burden and determinant of malnutrition in children with several types of congenital heart disease (CHD).Methods:This is a descriptive cross sectional study of children attending the outpatient clinic of UNTH, Ituku – Ozalla, Enugu State, over a six year period from March 2007 to April 2014. Data analysis was done with Statistical Package for Social Sciences (SPSS) version 19 (Chicago IL).Results:Forty thousand one hundred and twenty three (40,123) children attended the outpatient clinic during the study period. Of these, 50 had congenital heart disease, from which 46 were found to have various degree of malnutrition, giving a prevalence of 92% among children with congenital disease and 0.11% in the general population. Malnutrition showed significant correlation between age in years, age appropriate dietary adequacy and pulmonary hypertension. (r= 0.22, p = 0.01; r = 0.20, p = 0.02; r = 0.15, p = 0.01).Conclusion:Children with CHD develop severe malnutrition and growth failure. The significant contributing factors are mean age at presentation and age appropriate dietary adequacy.