Anomalies of the inferior vena cava pose a great challenge to surgeons. Although uncommon, these congenital vascular malformations may have significant surgical implications. Awareness of their presence is essential to avoid inadvertent injury and major bleeding during retroperitoneal procedures. An accurate preoperative diagnosis and detailed planning play a crucial role to obtain successful outcomes when confronted with them. Several surgical techniques have been recommended to protect these anomalous venous structures. The aim of this review is to contribute to the knowledge of the most common types of anomalies of inferior vena cava encountered during retroperitoneal procedures. With this purpose, a summary of their anatomy, embryology, incidence, diagnosis and intra-operative management is presented.
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