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The pulmonary valve separates the right ventricle outflow tract from the pulmonary artery and prevents deoxygenated blood from the pulmonary artery to return into the right ventricle. The pulmonary valve is the most rarely diseased of all heart valves. Pulmonary valve stenosis is usually congenital, while pulmonary regurgitation most often occurs as a consequence of percutaneous or surgical treatment of isolated pulmonary stenosis or tetralogy of Fallot. Patients with pulmonary stenosis and pulmonary regurgitation may be asymptomatic for a long period of time, but will eventually show symptoms such as reduced exercise capacity and dyspnea. In pulmonary regurgitation patients experience palpitations when cardiac arrhythmias occur. In advanced cases of pulmonary stenosis or pulmonary regurgitation right-sided heart failure may occur due to pressure or volume overload of the right ventricle. The initial diagnostic test is transthoracic echocardiography. However, poor acoustic windows make the evaluation of the pulmonary valve more challenging than the evaluation of other valves. Therefore, additional imaging techniques, such as magnetic resonance imaging, are used for the assessment of the valve. Due to the distance between the transducer and the valve, transesophageal echocardiography is usually not superior in visualization of the pulmonary valve. Percutaneous pulmonary balloon valvuloplasty is the procedure of choice for the treatment of hemodynamically significant pulmonary stenosis, while pulmonary regurgitation is usually treated with pulmonary valve replacement.