Abstract: One of the primary causes of mortality and morbidity in children are congenital heart disease (CHD); they have become a major burden on the global health. Babies with congenital heart disease are at a higher risk to be born with low birth weight. The purpose of the study is to investigate the relationship between the presence of CHD and low birth weight in the Sudanese children. This was an observational, cross-sectional study, conducted at Ahmed Gasim hospital, recruiting children with CHD from June to November 2018. The study included 139 study participants; 116(83.5%) mothers were between the ages of 20 to 40, only 85 (61.2%) mothers had regular antenatal care. 96 mothers (69%) were multiparous, 11 mothers (7.9%) reported an illness during pregnancy such as Diabetes and Hypertension. 90 children (64.7%) had been born with low birth weight while 49(35.3%) were born with normal weight. There was statistically a significant difference between the mean birth weight for the study group (2.35 kg) and the mean birth weight in Sudan (3.04 kg). Of the CHD lesions 20.9% were classified as cyanotic heart disease while the remained classified as a cyanotic heart disease. With regard to cyanotic status, a significant difference in the birth weight was not found. In Sudan, the percentage of low birth weight was found to be higher in children with CHD; larger birth weight deficits were found in Sudanese children than in other populations.