We present a case of recurrent haemo-pneumothorax in a young female patient with previously undiagnosed Ehlers-Danlos syndrome (EDS). The patient presented with a spontaneous haemo-pneumothorax not associated with menstruation. Following further subsequent episodes, left lower lobectomy was performed. In the past, the patient had suffered recurrent atraumatic bilateral patella dislocations which were never fully investigated. Histology of the lung tissue revealed features suggestive of EDS. Haemothorax is a rare complication of type IV EDS. There are very few reported cases of pulmonary presentation of EDS type IV.