Dilated cardiomyopathy with ataxia syndrome (DCMA) is an autosomal recessive disease frequently characterized by heart failure in early childhood. Although globally rare, DCMA is common in the Hutterites of southern Alberta who represent the largest collection of patients in the world. Alberta Children’s Hospital investigators previously identified a single intronic G>C mutation in the poorly characterized gene DNAJC19 as being responsible for DCMA. In collaboration with Stanford University, we have generated induced pluripotent stem cell (iPSCs) from DCMA patient peripheral blood mononuclear cells. Differentiating iPSCs into beating cardiomyocytes (iPSC-CMs) creates a disease-, patient-, and tissue-specific in vitro model of DCMA. However, our current model system has limitations due to lack of appropriately matched controls. This thesis aimed to create isogenic controls from our patient-derived iPSCs using the clusters of regularly interspaced short palindromic repeats (CRISPR) and CRISPR-associated endonuclease 9 (Cas9) system. We hypothesized that repairing the G>C mutation in DNAJC19 of our patient iPSCs will produce iPSC-CMs with a phenotype comparable to healthy controls and introducing the G>C mutation into DNAJC19 of healthy iPSCs will produce iPSC-CMs with a DCMA phenotype. Although isogenic controls have yet to be derived, this thesis outlines a potential workflow for the genomic editing of DCMA iPSCs. Our approach utilizes the use of an RNP-complex system that is delivered to iPSCs via lipofection using Lipofectamine Stem.