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The incidence of situs inversus totalis is low. Localization of symptoms and signs will change for the patient with this anomaly when complicating abdominal disease. One patient with cholecystolithiasis in our hospital coexistence with congenital heart disease and situs inversus totalis had abdominal surgery, and more attention should be paid to maintaining the stability of circulation during operation. In this case report we discussed treatment of this patient with situs inversus totalis.