Hemoptysis is an important respiratory symptom that may be fatal. Despite of the advanced diagnostic procedures, etiology of hemoptysis in some patients remains unclear. The partial pulmonary artery agenesis and systemic shunt was detected by us, in a 25 year-old female patient which has been hemoptysis with unknown etiology for 5 years. Symptoms of the patient was taken under control by embolisation procedure. The aim of this report is to take attention to this rare cause of hemoptysis and to emphasize that pulmonary angiography should be considered in the diagnosis of the hemoptysis with unknown etiology.