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Rectal neuroendocrine tumor is a rare disease that is difficult to diagnose by clinical and imageological examinations. The treatment of rectal neuroendocrine tumors is still controversial.A 50-year-old woman complained of abdominal pain beneath the xiphoid process for 1 day. Physical checkup revealed tenderness at the right upper abdomen. A fecal occult blood test was positive. MRI showed an occupation lesion in the right lobe of the liver. Colonoscopy examination showed a lesion at the lower rectum with an ulcerated surface that was tough in texture. No abnormality was found in the tumor markers.Rectal neuroendocrine tumor (G3) with liver metastasis.Neoadjuvant chemotherapy followed by laparoscopic surgery was given.The patient followed up regularly in the outpatient department for 13 months after surgery, and no sign of recurrence was found.Neoadjuvant chemotherapy followed by laparoscopic surgery is a new idea for the treatment of rectal neuroendocrine carcinoma with distant metastasis, which offers favorable conditions for saving the anus during the surgery to enhance the patient's quality of life.