Ebstein's anomaly is a rare congenital heart defect. Patients with severe symptomatic tricuspid regurgitation requiring surgical correction often have conduction system disease. We present a case of a 14 year-old girl with Ebstein's malformation and bioprosthetic tricuspid valve who required permanent pacing for symptomatic bradycardia. The placement of the right ventricular pacing lead was facilitated by the use of the Doppler pulmonary artery velocity time integral as a surrogate for stroke volume. This case demonstrates the importance of site-specific pacing and the utility of Doppler echocardiography to optimize lead placement and cardiac performance in patients with Ebstein's anomaly and advanced conduction system disease.