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The revelation of situs inversus totalis by a peritoneal syndrome is an extremely rare event. The association of this condition with sigmoid diverticulitis and agenesis of the vermiform appendix has not been described in the literature. This paper aims to present the first case of this type while screening the literature on this topic. The authors present the case of a sigmoid diverticulitis associated with situs inversus totalis and agenesis of the vermiform appendix, in a 44-year-old male patient. Because of abdominal pain located in the right iliac fossa, elevated temperature (38.2 C) and biological examinations, acute appendicitis was rather simulated and considered as a presumptive diagnosis. Diagnostic accuracy was achieved during laparoscopic exploration of the peritoneal cavity, which proved the coexistence of visceral transposition, appendiceal agenesis and sigmoid diverticulitis, usually noted as a rare finding …