Inflammatory fibroid polyps (IFPs) are rare benign tumors of the rectum. Mutation and activating platelet-derived growth factor receptor alpha (PDGFRA) contribute to tumor development. We present a case of IFPs in the middle rectum that mimic rectal cancer. A 65-year-old woman presented with the symptom of fresh blood in the stool and body weight loss of 6 kg in the preceding 3 weeks. A rectal polypoid tumor was noted upon digital examination. Sigmoidoscopy showed a middle rectal tumor measuring 3 × 2.7 cm with obstruction. Computed tomography (CT) scans of the abdomen showed a rectal tumor that had invaded the sacral bone and was associated with four enlarged lymph nodes greater than 1 cm. The radiological report suggested a diagnosis of rectal cancer with lymph node metastases. To remove the obstruction, the patient was initially treated with excision of the tumor and loop sigmoidal colostomy to the abdomen wall. Total mesorectal resection of rectal and sacral tumor followed 10 days later. Histopathological examination of the rectal and sacral tumor showed proliferation of vessels, fibroblast-like spindle cells, and mixed inflammatory cells, including the plasma cells and eosinophils. The spindle cells were diffusely positive to PDGFRA and were focal positive to CD34 and smooth muscle actin. Based on histopathological and immunohistochemical findings, the diagnosis of IFP is indicated. This was the first reported case of IFPs of the rectum presenting with lymph node enlargement and attachment to the sacrum mimicking rectal cancer.