A case with an Alpha-fetoprotein-producing (AFP-producing) carcinoma originating from the rectum was described. A 41-year-old man, who underwent a palliative surgery for rectal carcinoma, was diagnosed with occupying liver lesions and a remarkable AFP elevation (3484.61 ng/mL), and the AFP declined obviously 10 days after the palliative surgery. So we carried out a biopsy of the liver lesions. The histopathology was reported as low differentiation adenocarcinoma. The immunohistochemistry of the tumor cells via liver biopsy showed: Villin, CDX-2 was positive, Glypican-3 was partial positive, CK7, CK20, AFP, Hepatocyte were all negative. The initial histopathology was reported as an AFP-producing rectum adenocarcinoma with liver metastasis, which was a rare disease. So far, only 17 reports, none has been reported in China. Then, we summarize the characteristic of the disease: diagnosed with hepatic metastasis, raised serum AFP and a poor outcome, in addition to primary symptoms. This kind of disease is highly malignant. © Huazhong University of Science and Technology and Springer-Verlag Berlin Heidelberg 2013.