Symptomatic hypertrophic cardiomyopathy (HCM) is rare in infants. Therapeutic options are limited and include beta-blocker or calcium-channel blocker therapy or surgical myotomy/myectomy. Atrioventricular sequential pacing has been utilized as an alternative to surgery in symptomatic adults with HCM. We report our results with transvenous dual chamber pacing in an infant with symptomatic HCM.