Several studies have reported the possibility of an association between recent exposure to transfusion and development of necrotizing enterocolitis (NEC). Our objective was to systematically review and meta-analyze the association between transfusion and NEC (TANEC), identify predictors of TANEC, and the assess impact of TANEC on outcomes. Medline, Embase, CINAHL, and bibliographies of identified articles were searched for studies assessing association with recent (within 48 hours) exposure to transfusion and NEC. Two reviewers independently collected data and assessed the quality of the studies for bias in sample selection, exposure assessment, confounders, analyses, outcome assessments, and attrition. Meta-analyses were performed by using random effect model, and odds ratio and 95% confidence interval were calculated. Eleven retrospective case-control studies and 1 cohort study of moderate risk of bias were included. Ten case-control studies had NEC not associated with transfusion as control patients (unmatched). Recent exposure to transfusion was associated with NEC. Neonates who developed TANEC were younger by 1.5 weeks, were of 528 g lower birth weight, were more likely to have patent ductus arteriosus, and were more likely receiving ventilatory support. TANEC infants had higher risk of mortality. Two pre-post comparative studies of 20 patients reported reduction of TANEC after withholding feeds during transfusion. Recent exposure to transfusion was associated with NEC in neonates. Neonates who developed TANEC were at overall higher risk of NEC. TANEC patients were at higher risk of mortality, but additional studies adjusting for confounders are needed.