Pulmonary sequestration is a portion of pulmonary tissue that does not have communication with the tracheobronchial system and is supplied by anomalous systemic arteries. Association of the pulmonary sequestration with congenital anomaly is not rare. However, to date, pulmonary vascular anomaly association has not been reported in the literature. Herein, we presented a 20-year-old man having intralobar sequestration associated with pulmonary vascular anomaly and high pulmonary artery pressure which decreased significantly after right lower lobe resection. (Tur Toraks Der 2009; 11: 124-6)