Background: Sinus of Valsalva aneurysm is an uncommon anomaly of the aorta. It occurs most frequently in the right sinus of Valsalva. Complications depend on its size and location. Situs inversus totalis is a rare condition wherein organs are reversed from their normal positions (mirror image).
Case presentation: We report the case of a 69-year-old man who presented situs inversus totalis known since his childhood and a sinus of Valsalva aneurysm in the right coronary sinus discovered by echocardiography following a history of infection. This was confirmed by CT-scan and MRI. Valve sparing surgery was performed using the remodeling technique associated with external aortic annuloplasty
Conclusions: The remodeling technique with exteranl aortic annuloplasty is usual technique to treat SVA.