RESUMO
Anomalies of the inferior vena cava (IVC) are often discovered incidentally, yet they pose significant challenges in both diagnosis and treatment. This study discusses a case of a 50-year-old female with situs inversus and well-differentiated cholangiocarcinoma, emphasizing the rarity and complexity involved. Imaging studies confirmed the presence of dextrocardia, abdominal situs inversus, and an interrupted IVC with continuation to the azygos vein. A thorough understanding of IVC embryology is crucial, as it helps explain the pathogenesis of such anomalies, which arise from disturbances in the embryonic venous system leading to various anatomical variations. Managing an interrupted IVC is critical due to the potential for vascular complications, highlighting the need for preoperative awareness. This study underlines the importance of recognizing and properly managing these rare vascular anomalies to improve patient care.