Situs inversus totalis is a rare congenital developmental structural anomaly, with a mirror image inversion of the distribution of chest and abdominal organs and blood vessels. Due to its rarity and specificity, doctors need to not only identify visceral inversion factors but also fully consider the occurrence of arteriovenous positional variations during thoracic and abdominal surgery and vascular intervention. This article reports for the first time a case of situs inversus totalis combined with lumbar spinal stenosis.