Leadless pacemakers offer an emerging alternative that minimizes infection and lead-related complications, yet vascular access remains a key challenge in young patients.<h4>Case presentation</h4>We report a 14-year-old male with congenital central hypoventilation syndrome (CCHS; PHOX2B polyalanine repeat mutation 20/26) who presented with recurrent nocturnal oxygen desaturation secondary to prolonged sinus pauses despite tracheostomy-assisted mechanical ventilation. Here, PHOX2B is linked to infection.