A 36-year-old man visited his local hospital because of abnormal findings on chest X-ray at his annual medical check-up. He was referred to our hospital because chest computed tomography (CT) revealed multiple nodules on the right upper and lower lobes of the lung. The bronchoscopic biopsy specimens from nodules of the right upper lobe suggested intraluminal organization and prior hemorrhage. We suspected Ehlers-Danlos syndrome (EDS) from his medical history of repeated pneumothorax and subcutaneous hemorrhage, and family history. We therefore performed genetic and biochemical analysis using blood and skin specimens. It revealed a decreased production of type III collagen from the patient's cultured fibroblasts and mutations in COL3A1. Thus, we confirmed the diagnosis of vascular EDS. Vascular EDS should be included in the differential diagnosis of cases with repeated pneumothorax or spontaneously reducing nodular shadowing of the lung.