Isolated supravalvular pulmonary stenosis is a rare finding, usually evidenced in the pediatric age. Here, we report a case of a 76-year-old patient with isolated, severely obstructive, pulmonary supravalvular stenosis. The peculiarity of the present case is the paucity of symptoms and lack of changes in right heart sections, despite of the importance of stenosis. The report confirms the utility of transthoracic two- and three-dimensional echocardiography and transesophageal echocardiography in the detection and quantification of this rare anomaly.