PURPOSE: Intrauterine growth retardatation(IUGR) is very important because of high mortality and morbidity in the neonatal period. We studied the intrauterine growth retardation pattern in neonates with congenital heart disease(CHD). METHODS: One hundred seventeen cases with CHD(acyanotic, 73 cases; cyanotic, 44 cases) who had no other congenital malformation or maternal diseases that might affect fetal growth were enrolled in this study along with 120 control cases without CHD. We analyzed birth weight, crown-heel length and neonatal ponderal index. RESULTS:The proportion of IUGR infants was 17 out of 237 cases(7.2%). Compared to a normal control group(5/120, 4.2%), the CHD group had more IUGR(12/117, 10.3%)(P=0.006). Low birth weight(LBW) was higher in the CHD group(26/117, 22.2% vs 15/120, 12.5%; P=0.048). Among the CHD group, acyanotic CHD had often more than cyanotic CHD(21/73, 28.8% vs 5/44, 11.4%; P= 0.028). The proportion of IUGR among the LBW was 12 out of 41 cases(16.3%) in total. But, there were no significant difference in the proportion of IUGR among the LBW(10/26, 38.5% vs 2/15, 13.3 %; P=0.089), the proportion of IUGR among the prematurity(3/25, 12.0% vs 2/25, 8.0%; P=0.637) and abnormal neonatal ponderal index(5/12, vs 3/5; P=0.490). The proportion of short crown-heel length was high in the CHD group(7/117, 6.0% vs 1/120, 0.8%; P=0.028). CONCLUSION: Neonates with CHD had greater incidence of LBW, short crown-heel length and IUGR compared to normal neonates. Moreover, symmetrical IUGR was more common in IUGR neonates with CHD. Therefore, intrauterine development might be more influenced by intrinsic fetal factors than hemodynamic alteration of the circulation in CHD with IUGR.