Introduction. Unilateral absence of pulmonary artery is a rare vascular malformation. Because of this anomaly, the lungs are supplied by the system of collateral arteries. Case outline. We present a case of the right pulmonary artery agenesis in a female patient. She was admitted to the hospital because of hemoptysis. A computed tomography scan revealed a congenital malformation – the right lung was smaller in size, the right principal pulmonary artery had not been developed along with aberrant tortuous blood vessels. Conclusion. Symptomatic therapy was applied in the case of our patient. There was no need for any surgical treatment. However, in case of massive hemoptysis embolisation or lobectomy/ pneumonectomy will probably be applied.