Hemangioblastoma of the central nervous system (HB, hemangioblastoma) is a rare tumor of the central nervous system that occurs in adults, mostly in the cerebellar hemispheres or brainstem, accounting for about 2% of cases. Most of them are sporadic, and a few are familially inherited – VHL disease. With the popularization of microscopic techniques and improved diagnostic imaging, more and more HB patients are being detected. However, atypical HB is still easily misdiagnosed preoperatively as glioma or auditory neuroma, etc., and the risk of surgery for substantial HB is easily underestimated by the operator. HB is mostly seen in the cerebellar hemisphere or spinal cord. HB occurring in the cerebellar hemisphere is also known as Lindau tumor. A small number of combined retinal hemangioblastomas or pancreatic cysts are called VHL disease, which runs in families. Clinically, we see mostly disseminated HB, mostly in adults, slightly more males than females, with peak incidence at 25-29 and 40-44 years of age. We believe that the imaging is mainly divided into types: the first type is the most frequent, which is monocystic with single tumor nodule type with a total of 40 cases, the diameter of the cystic cavity is mostly 2.5-4.5 cm, and the diameter of the tumor nodule is mostly below 2 cm. The second type was the parenchymal type with 8 cases, all of which were located near the midline, and the diameter of tumor nodules ranged from 2.5-5 cm. The third type was the cystic solid type (at least 2 cystic cavities or more) with 6 cases. The fourth type was the least common, the multiple nodule type, with 4 cases. It can be single cyst with multiple nodules or multiple single cyst with single nodules, etc. The cystic part of the tumor showed hypodensity on CT, and the tumor nodes showed equal or high density. The typical cystic part of single capsule with single tumor nodule type MRI showed long T1 and long T2, and the tumor nodule showed equal T1 and equal or long T2 signal, and the enhancement was uniform and obvious with clear boundary. There is no obvious edema around the lesion. Parenchymal and a small proportion of cystic solid HB had abnormal and obvious enhancement, and T2 could sometimes see the vascular flow signal, indicating rapid blood flow, while preoperative DSA angiography could see obvious staining, which could clarify the blood supply artery and necessary embolization treatment. In contrast, most cystic solid HB cavities are variable in size and irregular in morphology, with reinforcement of the cyst wall and no surrounding edema reaction. The typical monocystic with monotumor nodular HB located in the cerebellar hemispheres is basically not misdiagnosed and is characterized by large cysts and small nodules with uniform and non-enhancing cyst walls, while the nodules are uniformly and significantly enhanced with no surrounding edema and clear borders. The main differentiation: (1) Cystic glioma: mainly hairy cell glioma, the cyst wall can be unevenly thick and thin, the tumor nodules are unevenly sized and less enhanced than hemangioblastoma, the cyst wall can be enhanced and there is edema in the periphery. Immunohistochemistry was positive for GFAP and negative for NSE. In contrast, HB is negative for GFAP and positive for NSE. (2) Auditory neuroma: If the multicystic type located in the pontocerebellar horn is easily misdiagnosed as auditory neuroma, but there is usually no manifestation such as enlargement of the internal auditory tract and progressive hearing loss. (3) Angiomatous meningioma: It presents as a substantial mass associated with the meninges. Parenchymal HB with abnormal and obvious enhancement and no peripheral edema is usually not related to the meninges and can be easily distinguished from others such as meningioma or metastatic carcinoma.