Sarcomeric cardiomyopathies are cardiovascular disorders that are caused by defects in genes encoding sarcomeric proteins. Depending on the location of the mutation within the gene, the disease phenotype manifests in different types of cardiac disorders, including hypertrophic and dilated cardiomyopathy, although mutations within the same gene can result in different phenotypes. Additionally, variability in penetrance and expressivity may result in a heterogeneous disease phenotype, not only across unrelated families but also within the same family. Hence, studies are needed to unravel both the initial and progressive changes initiated by sarcomere mutations, which will aid in better understanding how mutations in the same gene can lead to different phenotypes. As heart samples of the patients are poorly available, generation of cardiomyocytes from patient-specific induced pluripotent stem cells (iPSCs) provides a unique opportunity to investigate the pathophysiology of the mutation and to gain new insights into the underlying mechanisms. In this chapter, we review the emerging knowledge within the growing field of modeling sarcomeric cardiomyopathy with human iPSC-derived cardiomyocytes. These iPSC-based studies employed diverse systems ranging from single cell techniques to 3D engineered heart tissue. Where possible, we also compare the results of different studies with patient phenotypes. Furthermore, we discuss the prospects and limitations of the current state-of-the-art iPSC technology. We conclude that application of patient- and disease-specific iPSCs and differentiation into cardiomyocytes certainly offers the advantage to recapitulate the disease phenotype and will continue to provide critical knowledge about the underlying mechanisms of sarcomeric cardiomyopathies. This may allow development of disease- and patient-specific drug therapies for these devastating diseases to improve cardiac function and to prevent progressive cardiac remodeling.