Two patients with partial absence of the right and left pulmonary veins respectively are described. Congenitally absent pulmonary veins are a rare phenomenon and the diagnosis is often missed or delayed. When children present with haemoptysis and/or recurrent respiratory infections with no obvious underlying cause, absence of the pulmonary veins must be part of the differential diagnosis. Special investigations to be done in these patients are chest roentgenograph to compare the lung volumes, radio-isotope scan, echocardiography and cardiac catheterisation. Magnetic resonance imaging may be helpful in defining the cause and site of obstruction of the pulmonary veins. Depending on the cause, surgery might be considered.