Wilson’s disease is a rare autosomal recessive disorder of copper metabolism caused by mutations in the ATP7B gene. A 4-year-old boy, born of a consanguineous marriage, presented with recurrent daily syncopal attacks. Initial cardiac evaluation showed frequent polymorphic premature ventricular contractions and non-sustained ventricular tachycardia on Holter monitoring. Despite beta-blocker therapy, episodes persisted. Persistently elevated liver enzymes prompted hepatology referral. A liver biopsy suggested metabolic liver disease, and whole-exome sequencing confirmed Wilson disease with a homozygous ATP7B mutation.

This case illustrates a rare presentation of Wilson’s disease, as ventricular arrhythmia-induced syncope was the initial and only manifestation in a young child before the development of classic hepatic or neurological signs. It underscores the importance of considering metabolic and genetic aetiologies in paediatric arrhythmias, especially when accompanied by abnormal liver function. Moreover, early recognition and initiation of chelation therapy can prevent disease progression and enable timely screening and management of affected family members.