BACKGROUND Neurocysticercosis is a parasitic infection that commonly affects the ventricles, subarachnoid spaces, and spinal cord of the central nervous system. The authors report an unusual manifestation of purely posterior fossa neurocysticercosis treated with endoscope-assisted open craniotomy for resection. OBSERVATIONS A 67-year-old male presented with 2 months of progressive dizziness, gait ataxia, headaches, decreased hearing, and memory impairment. Imaging revealed an extra-axial cystic lesion occupying the foramen magnum and left cerebellopontine angle with significant mass effect and evidence of early hydrocephalus. Gross-total resection was accomplished via a left far lateral craniotomy with open endoscopic assistance, and pathological findings were consistent with neurocysticercosis. Postoperatively, he was noted to have a sixth nerve palsy, and adjuvant therapy included albendazole. By 9 months postoperatively, he exhibited complete resolution of an immediate postoperative sixth nerve palsy in addition to all preoperative symptoms. His hydrocephalus resolved and did not require permanent cerebrospinal fluid (CSF) diversion. LESSONS When combined with traditional skull base approaches, open endoscopic techniques allow for enhanced visualization and resection of complex lesions otherwise inaccessible under the microscope alone. Recognition and obliteration of central nervous system neurocysticercosis can facilitate excellent neurological recovery without the need for CSF diversion. more...