Neurocysticercosis is an infection caused by the larvae of the pork tapeworm Taenia solium. Parenchymal lesions commonly present with seizure activity and intraventricular lesions can cause hydrocephalus. A 33-year-old female patient presented in a comatose state with acute hydrocephalus and a fourth ventricle lesion. She underwent placement of an external ventricular drain. Resection of the fourth ventricle lesion through a suboccipital approach allowed for restoration of normal cerebrospinal fluid (CSF) flow and relief of midbrain compression. The lesion was resected intact and the patient returned to normal neurological function. No CSF diversion procedure was necessary. The patient was discharged on cysticidal and steroid therapy. We concluded that surgical resection of lesions in the fourth ventricle attributed to neurocysticercosis is appropriate when brainstem compression is prominent. Resection may also avoid the need for permanent CSF diversion. We also reviewed the evidence-based management strategies described in the literature.