Abstract:  Objective To investigate the clinical characteristics of children with hemoptysis of pulmonary vascular abnormalities and to explore the diagnostic and therapeutic methods in perioperative period. Methods The clinical data and image features of 16 children with hemoptysis of pulmonary vascular abnormalities were retrospectively analyzed. The treatment plan and the effects were followed up and evaluated. Results There were 4 males and 12 females with 3 cases aged <1 year old, 13 cases aged 5 to 13 years old. Angiography confirmed that there were 13 cases (81.25%) of primary pulmonary collateral circulation, 1 case (6.25%) of pulmonary arteriovenous fistula, 1 case (6.25%) of bronchial artery pulmonary vascular fistula, and 1 case of unconfirmed type. Eleven cases received a catheter based therapy, one received abnormal vascular ligation under thoracoscope, one received the resection of part of the lung lobe, and 3 cases gave up the therapy. Among those 11 cases who received a catheter based therapy, 2 cases showed a little shunt by radiography immediately after the therapy, 3 cases showed recurrent hemoptysis within one year of therapy, all of these were aortic collateral circulation. Two cases treated by surgical had no recurrence. Conclusions Pulmonary vascular abnormality is one of the main causes of hemoptysis in children, and the main types was main pulmonary collateral circulation. Catheterization is still the first-line treatment for children with hemoptysis of pulmonary vascular abnormalities.