A 47-year-old woman was admitted for evaluation of exertional dyspnea (NYHA II-III) that was lasting for 20 years. On physical examination, a grade III systolic murmur in the left second intercostal spaces was detected. The chest Xray showed mild cardiomegaly. ECG showed biatrial and biventricular hypertrophy. Transthoracic echocardiography showed severe infundibular pulmonic stenosis (pressure gradient=174 mmHg), moderate tricuspid regurgitation (jet velocity=6.6 m/sec) with biatrial enlargement and biventricular hypertrophy. Transesophageal echocardiogram showed severe infundibular pulmonic stenosis. Right ventriculography and catheterization revealed a pressure gradient between pulmonary artery and right ventricle (178/6 mmHg). We reported a rare case of infundibular pulmonic stenosis with intact ventricular septum in adult with literatures.