Necrotizing enterocolitis (NEC) is a relatively rare but devastating entity associated classically with the preterm cohort in the neonatal intensive care unit. Preterm and term babies with congenital heart disease are at risk of a number of comorbidities because of the hemodynamic derangements due to a structurally abnormal heart and the corrective procedures adopted. Necrotizing enterocolitis is one of the dreaded complications associated with this cohort and impacts the course of these babies in the hospital in a major way. A large majority of term babies with NEC are in the backdrop of a significant congenital cardiac lesion. This review article summarizes the literature and elaborates this entity including its specific features, risk factors associated with its causality, histopathology and related aspects of hemodynamics, and feeding in this vulnerable population. It also provides insight into modifiable risk factors and early markers of detection of gut necrosis to facilitate prevention and early detection. It highlights the subtle but definite difference in outcome variables to help physicians enable the parents of babies with heart disease to develop a better understanding of the entity and its expected course while counseling.