A neuroendocrine carcinoma of the anal canal is a very rare entity; however, this type of tumor is known for its aggressive progression and poor prognosis. We describe the case of a 58-year-old female with a neuroendocrine carcinoma arising in the anal canal. The tumor was found in the anal canal with multiple liver metastases. The patient died due to massive liver metastases 11months after diagnosis and operation. For its rarity and clinical significance, we report the case with a review of the literature.