Patients with a double inlet ventricle may undergo surgery using a modified Fontan procedure, in which the pulmonary ventricle is not utilized, or a procedure in which a pulmonary ventricle is created through ventricular septation. Ventricular septation is preferred to the Fontan procedure because there is better cardiorespiratory response to exercise after surgery. A 4-year-old girl with Holmes heart underwent ventricular septation on 12 May 1998. Pulmonary artery banding had been performed at 3 months of age and rebanding 16 days later. She was well and continued to grow. Ultrafast computed tomography and cardiac catheterization prior to surgery showed a double inlet left ventricle (LV) connected to a right posterior aorta with a right-sided rudimentary right ventricle that drained to the left anterior pulmonary trunk. Left ventricular end diastolic volume was 218% of normal and the ejection fraction was 79%. After debanding and enlargement of the bulboventricular foramen, a 3 x 4-cm composite patch of equine pericardium and Dacron velour was used to septate the ventricle, with transmural stitching at the apical portion. The patient survived the operation with complete atrioventricular block, and was extubated 6 days later. A permanent pacemaker was implanted 1 month later. One year after surgery, she was doing well. Echocardiography revealed paradoxical septal motion with good ventricular function. This is the first report of successful ventricular septation of a double inlet left ventricle performed in Taiwan.