This article reports a 16-year-old patient with severe pulmonary valve regurgitation after corrective surgery for tetralogy of Fallot. The shape of the right ventricular outflow tract to the main pulmonary artery was cone-shaped, which is extremely challenging. After admission, percutaneous pulmonary valve replacement with self-expanding valve was successfully performed. The patient’s condition remained stable during the 2-year follow-up period after surgery. This case aims to provide a reference for percutaneous pulmonary valve replacement in patients with cone-shaped right ventricular outflow tract.