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Introduction: Colorectal cancer is one of the most common types of malignant tumors worldwide. In patients with situs abnormalities such as situs inversus totalis or situs ambiguus, the presence of this tumor could be a challenge for the surgeon, especially in cases in which the laparoscopic approach is considered. Case presentation: We report the case of a 69-year-old male patient with situs inversus totalis. This particular case of situs inversus totalis was not a classical type because the patient had bilateral bilobed lungs, polysplenia, preduodenal portal vein in association with midgut malrotation. The pathology report after surgery revealed moderately differentiated adenocarcinoma of the sigmoid colon, stage pT3 N1c M1a, liver metastases but without metastases in the eight resected lymph nodes. We compared this rare association of diseases of particular anatomic aspects with other reports in the specialty literature. Conclusion: The identification of situs abnormalities or other malformations in patients with resectable colorectal cancer is essential, thus preoperative imaging studies are imperative for a proper surgical management. Colorectal cancer metastasizing patterns in patients with intestinal malrotation need to be further investigated.