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Objective To establish a birth rate for catecholaminergic polymorphic ventricular tachycardia (CPVT) diagnosed in childhood and observe trends in presentation and management.Design Retrospective cohort study.Setting The Inherited Arrhythmia Clinic at The Sydney Children’s Hospitals Network, a paediatric tertiary referral network, New South Wales (NSW), Australia (2002–2021), where there are 86 000–97 000 live births/year.Patients Children diagnosed with CPVT aged 0–16 years.Interventions Clinical data were extracted and evaluated for trends. Using birth year data, the birth rate of CPVT detected in childhood was calculated.Main outcome measures Birth rate of CPVT detected in childhood in NSW (with post hoc comparison to New Zealand), trends in diagnosis and management, and outcome at last follow-up.Results 32 children in NSW were diagnosed with CPVT between 2002 and 2021 (0–16 years, median 9 years, 14 (54%) female). Of these, 28 (88%) presented with symptoms (cardiac arrest 20/32, 62.5%) and four (12%) were identified through family screening. Relevant genetic variants were identified in 25/31 (78%). During follow-up (median 4.5 years), symptomatic cardiac events (death n=1) occurred in 10 (33%), largely related to suboptimal adherence or monotherapy beta blocker. In NSW, CPVT was diagnosed during childhood following 1 in 65 000 live births (95% CI 1 in 91 000 to 1 in 46 000). In New Zealand, the corresponding figure was 1 in 84 000 live births (95% CI 1 in 138 000 to 1 in 52 000).Conclusions The rate of infants born who are later diagnosed with CPVT in childhood is approximately 1 in 65 000 live births. Suboptimal adherence and beta blocker therapy without flecainide appeared related to recurrent cardiac events.