Introduction: Data evaluating heart transplant (HT) outcomes in patients with genetic cardiomyopathies is limited. Cardiac features are prominent in these genetic disorders, resulting in progressive heart failure that may be lethal without cardiac transplantation. However, no single center has accumulated enough experience to investigate post-HT outcomes for these rare cardiomyopathies. The primary aim of this review is to synthesize literature investigating if HT is a viable treatment option for genetic cardiomyopathies including Duchene Muscular dystrophy (DMD), Becker’s (BMD), Limb-Girdle (LGMD), Emery Dreifuss (EDMD); mitochondrial disorders, Friederich’s ataxia (FA), Barth’s disorder and fatty acid oxidation disorders (FAOD). Methods: We performed a systematic literature search of PubMed, EMBASE, and Google Scholar identifying studies reporting HT outcomes published up to June 2021. Results: A total of 42 articles were selected for review with 118 patients reported. Patient data with age at HT, gender distribution, mean follow-up, reported survival data, and post-operative outcomes are reported in Table 1. Patients with DMD, BMD, LGMD, and EDMD had HT during the second and third decade of life and were reported to have favorable survival outcomes at one and five years. In FA, 11 patients underwent cardiac transplantation at mean age 21.2 years with reported survival of 100% at one and five years. Patients with Barth syndrome received HT at younger ages with mean age 1.6 years. Mortality in these patients as well as mitochondrial disorders were low even with longer reported follow-up time. Cardiac transplants in FAOD were rare with one female receiving HT at the age of 27 months and was reported to be complication-free after 12 years. Conclusions: This review suggests that post-transplantation outcomes in genetic cardiomyopathy patients with multisystem involvement are generally favorable, with high survival and few post-operative complications.