A 23-year-old female patient, with no known past medical history, was referred to our centre at the 9th week of her first pregnancy because of incoercible vomiting and mild dehydration. She was admitted to the Obstetrics department, where she received symptomatic treatment with antiemetics and prokinetics. She was discharged on the third day with the diagnosis of hyperemesis gravidarum. Nevertheless, 2 weeks later, the patient was readmitted for presenting incoercible vomiting, dizziness, and headaches. On this occasion, vitamin B6 was added to her treatment, and she was discharged on the fifth day. On the 16th week of gestation, the patient was readmitted because she persisted with the symptoms. At this time, the patient was obtunded and disoriented, and she also complained of an oppressive occipital headache. On physical examination, left VI cranial nerve palsy, papilledema, and right ankle clonus were found. Brain magnetic resonance imaging (MRI) was performed, suspecting a dural sinus thrombosis. However, the study revealed triventricular hydrocephalus with transependymal edema due to a tumor in the posterior fossa. The patient was assessed by the authors, and she underwent an urgent ventriculoperitoneal shunt with a high pressure valve without any complications. In spite of a favorable course in the immediate postoperatory period, the patient experienced neurological decline with blurry vision, vomiting and headaches 10 days later. These symptoms did not respond to medical treatment with dexamethasone. In view of the latter, an endoscopic third ventriculostomy was performed, and her derivative system was removed. At the same time, we discussed with the Obstetrics department the need to operate on the mass, since the patient showed progressive involvement of the brainstem. At 22 weeks of gestation, the patient underwent a right sub-occipital craniotomy with partial resection of a highly vascularized mass in the posterior margin of the foramen magnum. Its intraoperative biopsy reported a low-grade stromal tumor, suggestive of hemangioblastoma. The surgery was performed without any obstetric or neurologic complications. In the postoperatory period, the patient only presented mild right hemiparesis, which remitted within a few days. The definitive biopsy showed the presence of a vascular neoplasm with irregular “stag-horn” cells, and positive immunohistochemistry for CD34 and vimentin. All of the latter was compatible with hemangiopericytoma. The patient continued to be followed at the Neurosurgery and Obstetric outpatient clinics after the 26th week of gestation. She underwent a cesarean section at the 36th week because of intrauterine growth restriction, and it was performed without any complications. Currently, both the mother and the baby are in good health.