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We describe a case of a 44-year-old male with a history of Wolf-Hirschorn syndrome (WHS) with seizures and mental retardation who was evaluated for what was thought to be a seizure. He was found to be severely bradycardic with a heart rate of 24 bpm. The electrocardiogram revealed third-degree atrioventricular block and he subsequently underwent an uncomplicated single-chamber pacemaker implantation procedure. This is a unique report given its status as the first described case of bradycardic rhythm abnormalities in a patient with WHS.

A Different Breed of Wolf: First Known Case of Wolf–Hirschorn Syndrome with Third-degree Atrioventricular Block Requiring Pacemaker Implantation