A case of renal cell carcinoma in a patient with situs inversus totalis is reported. Only 8 cases of malignant neoplasms have been reported in situs inversus totalis, and our case represents the second case report of renal cell carcinoma in a patient with situs inversus totalis. The frequency of situs inverses totalis is between I in 8,000 and I in 20,000. Cardiac and pulmonary anomalies are common in patients with situs inversus totalis. Renal anomalies, including agenesis, dysplasia, hypoplasia, ectopia, polycystic kidney, and horseshoe kidney, have been reported. Because of the association between situs inversus and cardiac, pulmonary, renal, and vascular anomalies, management of the patient with situs inversus and urologic disease requires careful preoperative evaluation.
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