AbstractNeural crest cells appear on top of the neural folds at closure of the neural tube. These cells are capable of migrating and differentiating into multiple tissues. In 1983, Kirby et al., using chimera methodology, reported that cranial neural crest cells participated in aorticopulmonary and truncal septation. Since that time, many experimental studies have been done in this field. In this paper, we report new experimental findings and review four areas of study on neural crest cells, i.e. their general characteristics, participation of neural crest cells in formation of the normal heart, cardiovascular anomalies due to ablation of the neural crest cells, and the relationship between neural crest cells and congenital malformation syndromes, especially the DiGeorge sequence.