Background. Dextrocardia with situs inversus is a rare congenital condition with less than 1 person in 10 000, in wich the internal organs are mirrored inside the human body. The majority of the patients with dextrocardia and situs inversus are phenotypically normal and have a normal life without any complications related to their congenital condition. About 5 10% of the these patients develop another congenital defects. There are only few published cases of the patients with situs inversus with dextrocardia associate d with aortic valve regurgitation. Case report. The 33 years old male with dextrocardia and situs inversus diagnosed in the childhood was consulted during routine medical examination. Chest radiography showed dextrocardia and situs inversus. The electroca rdiogram showed sinus rhythm with right axis deviation and reverse R wave progression in the precordial leads. He was examined by transthoracic echocardiography and third degree aortic regurgitation was found, moderate dilatation of the sinus of Valsalva 43 mm, and no dilatation of the ascending aorta 35 mm. There were no data for aortic dissection. The ejection fraction of the left ventricle was 55%. Computer tomography (CT) showed reversed positioning of mediastinal and abdominal organs complete sit us inversus and dextrocardia. On CT there were no signs of stenosis or dissections of the thoracic and abdominal aorta. The patient was referred to cardiac surgery for correction of valvular pathology. A complex aortic valve repair was performed. Postopera tive period was without complications. On control echocardiography after one month there was no important aortic regurgitation.Conclusions. Dextrocardia with situs inversus and aortic valve regurgitation is a very rare cardiac pathology. If cardiac surge ry is necessary it can be challenging but feasible with good results.