Absent pulmonary valve is a variant of tetralogy of Fallot, characterized by small or rudimentary, stenotic or regurgitant pulmonary valve with dilated pulmonary artery. It is uncommonly associated with double-chamber right ventricle. We hereby report a case with preoperative diagnosis of ventricular septal defect with pulmonary stenosis based on routine transthoracic echocardiography. Intraoperatively, transesophageal echocardiography showed a rare combination of double-chamber right ventricle in association with ventricular septal defect and absent pulmonary valve, which was confirmed during surgery.