Background: Down syndrome (DS) is one of the commonest disorders associated with chromosomal abnormality with a huge medical and social cost. DS is associated with several phenotypes including congenital heart defects, the point of our discussion. DS individuals are affected by these phenotypes to a variable extent thus understanding the cause of this variation is a key challenge. In this review, we will talk about the common congenital cardiac disease running in patients with down syndrome. Objectives: We aimed to review the literature on cardiac defects associated with Down’s syndrome, along with the etiology, clinical features, evaluation, diagnosis, and management of these types. Methodology: PubMed database was used for articles selection, papers were obtained and reviewed. Conclusion: Down syndrome is associated with a variety of congenital cardiac defects. Proper knowledge of these defects with the management approach is essential. Due to advancements in technology, prenatal diagnosis is possible. Almost all these conditions are cured by surgical repair and carry an excellent prognosis.