Spinal filum terminale pial arteriovenous fistulas (FT PAVFs) are uncommon. Most FT PAVFs are located in the lumbar region; far fewer are located in the sacral region. Due to the rarity of sacral FT PAVFs, the precise surgical dissection and removal of these lesions are challenging. Here, we report an FT PAVF in the sacral region. The patient was a 45-year-old male who suffered from progressive weakness and numbness of the bilateral lower limbs; his symptoms gradually worsened. Digital subtraction angiography (DSA) showed an AVF at the sacral canal at the S3-4 level. Microsurgical treatment with intraoperative DSA was performed, and the FT PAVF was resected. After the operation, the patient gradually recovered. Follow-up magnetic resonance imaging revealed a recession in the dilation of the spinal cord venous plexuses. A literature review was also performed, and a total of 14 FT PAVFs of the sacral region were identified. The patients identified in the literature review had an average age of 58.9 ± 12.9 years, and 92.9% of the patients were male. Spinal cord edema was present in 85.7% of the FT PAVF patients. Regarding treatment, 64.3% of the FT PAVF patients underwent microsurgical resection, 28.6% patients underwent endovascular treatment, and 7.1% patients underwent a hybrid operation; good outcomes were achieved with all three methods. Therefore, FT PAVF of the sacral region is a unique lesion whose angioarchitecture needs to be identified carefully; prompt treatment is necessary, and microsurgery can yield good outcomes.