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Situs inversus totalis is the complete transpositioning of thoracoabdominal viscera into a mirror image of the normal configuration. Choledochal cyst is the congenital cystic dilation of the biliary tract. Both these conditions coexisting in a patient is extremely rare. We hereby present a case of type IC choledochal cyst in a patient with situs inversus totalis presenting with biliary sepsis secondary to choledocholithiasis. Also detailed are the management and operative strategies employed to deal with this rare entity.