Most thoracic surgeons overlook the fact that spontaneous pneumothorax is one of the pulmonary complications of connective tissue disorders such as Marfan or Ehlers-Danlos syndromes, and they consider it as primary. In the following report we describe a unique case of spontaneous rupture of the sigmoid secondary to a spontaneous recurrent contralateral pneumothorax in a young patient with undiagnosed Marfan syndrome. 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 pneumothorax and if so, to follow further diagnostic procedures to anticipate any other visceral complications.