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Rectal neuroendocrine tumor (NET) is a relatively rare tumor. Well-differentiated NETs (G1 and G2) rarely display distant metastasis at initial diagnosis. Currently, treatment for the primary lesions of rectal NETs with liver metastasis remains controversial. The liver metastasis was resected in local hospital. Transanal endoscopic microsurgery (TEM) has emerged as an effective minimally invasive surgery for local resection of lower rectal lesions. Herein, we reported the initial application of TEM to remove the rectal primary lesion in patients with low rectal NETs (G2) with giant liver metastases.The patient, a 45-year-old woman, was primarily diagnosed with hepatocellular carcinoma and underwent curative resection of a giant liver lesion in a local hospital. Nevertheless, the postoperative pathologic examination revealed that the lesion was an NET (G2). The colonoscopy then showed a nodule 1.4 cm in diameter, 4 cm above the anal verge, located on the anterior wall of the rectum. The biopsy revealed that the nodule was also an NET (G2). However, the patient did not consent to abdominoperineal resection based on concerns for quality of life.Rectal NET with liver metastasis.The patient underwent curative resection of liver metastasis. And, TEM was adopted to resect the primary tumor in rectum.The patient has been disease-free for 2 years with a good quality of life and presents no local recurrence in the rectum.TEM is an appropriate palliative operation for therapy of rectal NETs with distant metastases, especially for primary rectal NETs located in low rectal.