BACKGROUND Hemangioblastoma (HB) is a rare tumor, comprising about 2% of all intracranial tumors. Although it is a benign tumor, due to the abundant blood supply and its close relationship with adjacent cerebral blood vessels, surgical resection is difficult and may cause complications such as bleeding. If HB can be correctly diagnosed before surgery, complications can be avoided by methods such as vascular embolism before surgery. CASE SUMMARY A 51-year-old male patient was admitted to our hospital because of blurred vision in his left eye for 2 years. Ophthalmological examination revealed oculus dexter vision acuity of 1.0 and oculus sinister vision acuity of 0.6. His left vision had tubular visual field, while his right vision had a partial defect. Computed tomography and magnetic resonance imaging showed a mass lesion at the left anterior base of the skull, which could have been a meningioma. During the operation, the tumor was found to be located at the entrance of the left optic nerve tube, closely adhering to the left optic nerve and the blood supply was extremely abundant. The tumor was carefully separated and diagnosed as HB postoperatively after pathological examination. CONCLUSION A rare HB at the anterior skull base could be distinguished by its imaging features, which is essential to the surgical procedures.