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Introduction: Ehlers-Danlos syndrome (EDS) is an uncommon cause of hemoptysis and spontaneous hemopneumothorax. We present a case with recurrent hemoptysis who had bronchial artery pathology and anomalous pulmonary venous return associated with EDS. Case: Twenty-years-old female presented with recurrent hemoptysis for two years. Thoracic tomography was normal. Six months of antituberculosis therapy were given due to M. tuberculosis growth in sputum cultures. Ear-nose-throat examination revealed lesions in bilateral nasal conchae and lesions were cauterized. As the hemoptysis persisted, a diagnostic bronchoscopy was performed and revealed an active source of bleeding at the orifice of superior segment of the right lower lobe. Bronchial artery ligation was performed via right thoracotomy. Echocardiogram revealed mitral and tricuspid valve prolapsed. Ophthalmologic examination was performed due to atypical localization of pupils. Bilateral coloboma, nistagmus and ectopia lentis were found. Patient history was positive for spontaneous ecchymosis, premature loss of teeth, increased joint laxity. Genetic physicians found these findings to be consistent with EDS type 6. Skin and subcutaneous tissue biopsies were performed and the definitive diagnosis was made using electron microscopy, fibroblast culture and mutation analysis. A hereditary transmission could not be demonstrated. Pulmonary angiography revealed a tortuous course in distal branches of bronchial arteries and early venous return consistent with arteriovenous fistula. Bilateral bronchial artery embolization was performed. Patient9s hemoptysis regressed. Conclusion: In this care, EDS was found as an uncommon cause of hemoptysis.