A 63-year-old white male patient of Polish descent had a 3-month history of headaches, vomiting, and episodes of blurring vision, compatible with intracranial hypertension syndrome. On admission, he had papilledema, nystagmus, and lethargy. A T1-weighted magnetic resonance imaging (MRI) scan showed a low signal intensity lesion in the anterosuperior part of the posterior fossa in contact with the falcotentorial dura junction, displacing inferiorly the cerebellar vermis and compressing the aqueduct of Sylvius, producing obstructive hydrocephalus. The mass enhanced homogeneously with gadolinium-based contrast. Flow voids compatible with vessels were observed around the tumor.. Magnetic resonance angiography (MRA) showed the intense tumoral vascularization. MRI of the neuroaxis did not reveal any other lesions. Results from whole-body contrast-enhanced computed tomography (CT) scans were normal. His serum levels of alpha-fetoprotein, human chorionic gonadotropin, and carcinoembryonic antigen were normal. An urgent endoscopic third ventriculostomy (ETV) was performed to treat the hydrocephalus. After the ETV, our patient did well. Five days later, he was operated on for tumor removal. The surgery was performed in a semi-sitting position via an infratentorial supracerebellar approach. The tumor was a highly vascularized and well-circumscribed mass. The lesion was meticulously dissected from the surrounding tissue. The tumor was found to be separated from the superior aspect of the cerebellar vermis by a thick arachnoidal layer. Several small vessels feeding the tumor and coming from the tentorium were coagulated. The mass dramatically reduced its size, allowing en bloc resection to be performed. A pathological examination demonstrated a tumor characterized by a double component, vascular and cellular, with a network of small and delicate vessels that separated groups of large polygonal cells, with clear and vacuolated cytoplasm and small, oval hyperchromatic nuclei. A reticulin stain and CD34 immunostain readily delineated the complex capillary networks of the HBL (hematoxylin and eosin, Gomori, periodic acid–Schiff stain, and CD34 immunohistochemistry). There were no complications, and our patient had a complete recovery of his preoperative symptomatology. Postoperative screening using clinical, laboratory, ultrasound, and body imaging scans for the detection of VHL disease were negative. A long-term follow-up until 5 years post surgery was uneventful.