Spontaneous hemo-pneumothorax is one of the pulmonary complications of connective tissue disorders such as Ehlers-Danlos syndrome (EDS). Most thoracic surgeons overlook this fact and they consider it as primary. In the following report, we describe a unique case of spontaneous recurrent hemo-pneumothorax in a young patient with undiagnosed EDS. The aim of this presentation is to raise a high index of suspicion of every thoracic surgeon to include in his differential diagnosis, the connective tissue disorders in any case of spontaneous hemo-pneumothorax.