The patient was a 17-year-old male who had been diagnosed with vascular Ehlers-Danlos syndrome (vascular EDS) following surgery for bilateral pneumothorax. He had had recurrent episodes of pneumothorax, but all previous episodes improved with either observation or chest drainage alone. However, this time, because the air leak persisted even after chest drainage, pleurodesis with autologous blood was performed twice, successfully stopping the air leak. Due to the disease characteristics that cause connective tissue weakness, it is necessary to avoid invasive procedures, and the treatment options for refractory pneumothorax associated with vascular EDS are limited, but it was thought that the minimally invasive autologous blood pleurodesis was an effective treatment that could be performed safely.