Congenital heart disease is a common disease present in 1-2 % of newborn children. 75-80% of children with DiGeorge syndrome are affected by it. The most common defects are conotruncal anomalities, among which the most common are tetralogy of Fallot, pulmonary atresia with VSD, truncus arteriosus and interrupted aortic branch. The neural crest cells have the most important role in the development of outflow region of the heart, as well as the development of the third and fourth pharyngeal arches that are affected in this syndrome. Due to this fact, children with DGS also have aplasia or hypoplasia of the thymus, cleft palate, face dysmorphia and a disorder in the development of the thyroid and parathyroid glands. This disease is genetically caused by a deletion on the twenty-second chromosome, region 22q11.2. DGS is known to appear in following generations, so it is important to provide genetic counseling. It is neccesary to search for congenital heart defects in children with DGS, because they are, along with hypocalcemia, the most common causes of death. Catheterization of the heart is the best available diagnostic tool, but echocardiography is more frequently used because it is non-invasive and very simple method. Cardiosurgical therapy of PGS has advanced and thus decreased mortality in these children. Taking into consideration genetics and the various malformations that affect these children, it is necessary that experts from diffrent areas collaborate in order to offer the best possible care and thus raise the quality of patients' lives.