Twenty-six patients in infancy and early childhood with severe pulmonary valve stenosis and intact ventricular septum are reviewed. They were selected from a larger series of 112 patients with pulmonary stenosis of any degree, on account of early onset of symptoms and the severity of the stenosis proven by cardiac catheterization and angiocardiography, at operation or at necropsy. Our criteria for severity in this series were: presence of symptoms within the first two years of life; right ventricular and right atrial hypertrophy on electrocardiography; and right ventricular pressure equal to or higher than systemic blood pressure. The warning signs prompting valvotomy are deterioration of the following features: cyanosis and dyspnoea; congestive cardiac failure; tricuspid incompetence; cardiac enlargement and pulmonary oligaemia on radiograph; and right ventricular and right atrial hypertrophy on electrocardiography. The lives of 13 patients were saved by timely valvotomy. These patients are all well six months to six years after operation. Five patients died before any operation could be performed. Eight patients died within 48 hours of operation. Had some of these patients been operated on earlier the evidence indicates that they would have had a better prognosis. Therefore the importance of early recognition, prompt treatment, and emergency valvotomy, if necessary, is emphasized.Severe pulmonary stenosis with intact ventricular septum and normal aortic root is a much graver condition in the first two years of life than in the older age group. In infancy and early childhood this disease is fatal unless promptly diagnosed and treated by pulmonary valvotomy. Insufficient general appreciation of this condition, despite a few reviews since 1952 (Johnson and Johnson, 1952; Gibson, White, Johnson, and Potts, 1954;Mustard, Rowe, and Firor, 1960;Mustard, Jain, and Trusler, 1968;Luke, 1966;Gersony, Bernhard, Nadas, and Gross, 1967) We are discussing only the early severe group in detail because only in this group did fatalities occur. Of the late severe group 18 patients have undergone successful elective pulmonary valvotomy and are well; a further two patients await operation. There have been many reviews of pulmonary stenosis in this older age group (Brock,