Hypoplastic Left Heart Syndrome (HLHS) is a severe and rare congenital heart disease characterized by the underdevelopment of the left side of the heart, which is responsible for pumping oxygenated blood throughout the body. This underdevelopment results in an insufficient supply of oxygenated blood to vital organs and tissues. Current treatments for HLHS face significant challenges and typically involve complex surgical interventions that can only extend patient survival to a limited degree. Heart transplantation is another potential option, but it is constrained by the scarcity of donors and the risk of immune rejection. Induced pluripotent stem cells (iPSCs), derived from somatic cells, possess the remarkable ability to differentiate into any cell type within the three primary germ layers, making them a promising tool for tissue regeneration, disease modeling, and repair. However, despite their potential, the clinical application of iPSCs remains limited, primarily due to the risk of tumorigenesis. This review explores the role of iPSCs in advancing the understanding and treatment of HLHS, examining their cellular and molecular mechanisms, historical development, and their application in cardiovascular research. Additionally, we consider the major challenges and limitations associated with the use of iPSCs in the context of HLHS, underscoring the critical need for further research to enhance their therapeutic efficacy and safety.