Background: Spontaneous ruptures of the colon and rectum are very rareclinical entities and always require laparotomy. In such cases, despite meticulous exploration an underlyingpathology cannot be revealed and most reports in the world literature relate spontaneous colon ruptureswith unusual connective tissue disorders such as Marfan or Ehlers-Danlos syndrome. Connective tissuediseases affect the structure and the synthesis of elastin and type III collagen, resulting in varioussystemic disorders. Among the several pulmonary manifestations, spontaneous pneumothorax is observedin about 5% of patients expressing one of the above syndromes. Case Report: In the following report wepresent a unique case of spontaneous rupture of the sigmoid colon secondary to spontaneous recurrentcontralateral pneumothorax. Conclusions: The aim of this presentation is to raise the level of suspicionin every surgeon and gastroenterologist to include connective tissue disorders in their differentialdiagnosis in any case of spontaneous colon rupture and, if found, to follow further diagnostic proceduresin anticipation of any other visceral complications.

Keywords: Adolescent, Colon, Sigmoid - surgery, Follow-Up Studies, Marfan Syndrome - surgery, Pneumothorax - surgery, Rupture, Spontaneous, Time Factors, Tomography, X-Ray Computed