Unilateral pulmonary vein stenosis is a rare congenital anomaly. A case is described in a girl who first presented at the age of four years with recurring haemoptysis but in whom diagnosis was not established until she was 16 years old. Pulmonary angiography demonstrated a minimally hypoplastic right pulmonary artery, and the laevophase showed normal pulmonary venous return from the left lung, but none from the right. Surgical treatment was necessary because of life threatening haemoptysis, and pneumonectomy was required in the light of the findings at operation.