Background: Emery-Dreifuss Muscular Dystrophy (EDMD) is an X-linked recessive muscular dystrophy associated with FHL1 genetic variations. It presents with diverse phenotypes, including hypertrophic cardiomyopathy (HOCM), other myopathies, and vocal cord dysfunction. Case Presentation: A 13-year-old male presented with an inaudible voice and was diagnosed with bilateral cricoarytenoid joint (CAJ) fixation. Previous injection laryngoplasty was ineffective, and he underwent posterior glottic diastasis laryngoplasty. Postoperatively, the patient suffered unexpected cardiac arrest, requiring emergency extracorporeal membrane oxygenation (ECMO) support for several days. He was diagnosed with HOCM and ultimately required orthotopic heart transplantation. During his hospital stay, he was managed with a temporary tracheostomy and sustained lower limb ischaemia, necessitating above-knee amputation and the use of an artificial limb. Genetic testing confirmed a maternally inherited FHL1 mutation consistent with EDMD, with features including muscle weakness in a humero-peroneal distribution, later extending to scapular and pelvic girdle muscles. Since decannulation, the patient has maintained a stable airway with effective phonation and improved vocal parameters. Flexible nasendoscopy confirmed stable bilateral CAJ fixation, with effective residual mobility in the right vocal cord and maintained airway function. His dysphonia is minimal, and he has gone on to achieve tertiary university qualification and represented nationally in his sporting achievements. Conclusion: This case underscores the unusual relationship between laryngeal dysphonia and cardiac comorbidity in EDMD and highlights the importance of multidisciplinary care. Vigilance for cardiac complications, including HOCM, is critical, especially during anaesthesia. Collaboration with speech therapy and genetics teams is essential to optimise outcomes in this rare patient population.