Cardiac involvement may occur in many forms of muscular dystrophy (MD). While cardiac disease may progress to warrant heart transplantation (HTx), there may be contraindications related to extra-cardiac disease including pulmonary and skeletal muscle involvement that limit overall survival and impairs post-transplant rehabilitation efforts. This study describes the MD HTx experience at a single high-volume center. We examined the clinical characteristics and outcomes of patients with MD with heart failure (HF) (n=28), patients with MD status post HTx (n=20) and non-MD HTx control group (n=40) matched 2:1 for age at transplant, sex, listing status, and antibody sensitization. Patients with MD who underwent HTx had increased ventilator days (2vs. 1 days, p=.013), increased hospital length of stay (20vs. 12 days, p=.022), and increased discharge to inpatient rehab (60%vs. 8%, p<.001). By 1 year post HTx, patients with MD more often required assistive devices for walking (55%vs. 10%, p=.01). Nonetheless, post-HTx survival was similar at 1 year (100%vs. 97.5%, p=.48) and 5 years (95.0%vs. 87.5%, p=.36). Of the HTx recipients with MD, 95% were followed by a neurologist, 60% by a neuromuscular specialist as part of the Muscular Dystrophy Association Clinic at our center. Transplantation is a feasible option for patients with MD and advanced HF. MD patients who undergo transplantation may benefit from multidisciplinary specialized care to optimize MD-related morbidity.