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Situs inversus totalis (SIT) is a rare anatomical variation of the internal organs, and solid pseudopapillary tumor of the pancreas (SPTP) is a rare tissue type of pancreatic tumors, classified as benign or low-grade malignancy. However, to our knowledge, a patient with SIT and SPTP is extremely rare and has never been reported.We retrospectively analyzed a case of SIT with SPTP in a 45-year-old woman. The main complaints were abdominal pain and sensation of heaviness for 2 weeks. There was tenderness and a mass that could be palpated in the right upper abdomen.Heart ultrasonography (USG), chest x-ray, computed tomography (CT), and contrast-enhanced computerized tomography (CECT) revealed a mirror-image dextrocardia and inversion of all abdominal viscera and a space-occupying lesion in the pancreas tail. Abdominal computed tomography angiography (CTA) showed no obvious abnormality of artery. The diagnosis of SPTP was finally made by postoperative pathological examination.The patient underwent resection of the pancreatic body and tail and splenectomy via laparotomy to completely remove the tumor.The patient was discharged with specific discomfort on postoperative day 7. At the 1.5-year follow-up, she recovered without issue.Surgical resection remains the only effective treatment of SPTP. SIT with SPTP can be accurately diagnosed by heart USG, chest x-ray, CT, and CECT of the upper abdomen. Abdominal aorta CTA before surgery can decrease the injury risk of blood vessels.