A 36-year-old right-handed woman with a history of low-grade astroblastoma presented with a 2-month history of worsening left-sided weakness, neck pain, right upper extremity numbness, gait ataxia, blurry vision, nausea, dizziness, and fatigue. She previously underwent gross total surgical resection in 2002 followed by 6 weeks of radiation therapy in 2004 for progressive disease. Tumor progression in 2015 prompted subtotal resection followed by hypofractionated stereotactic radiosurgery for continued progression in 2016 and six cycles of temozolomide in 2017. The patient has a long-standing history of orthostatic hypotension, back pain, spasticity, and left foot drop. On physical examination, the patient had persistent horizontal nystagmus on bilateral lateral gaze, tongue deviation to the right, and left-sided hemiparesis. Upper and lower extremities were spastic bilaterally but worse on the left side. Lower extremities were deconditioned with 0/5 motor strength in the left ankle, which was hyper-reflexic with nonsustained clonus. Fine touch sensation was diminished in the right upper extremity and proprioception was diminished in the right upper extremity and both lower extremities. MRI imaging revealed a right paramedian intradural foramen magnum lesion, most likely representing recurrent expansile cervicomedullary astroblastoma given the patient’s history [-]. The expanded differential included astrocytoma, ependymoma, DNET, lymphoma, ganglioglioma, subependymoma, metastasis, lower cranial nerve schwannoma, or granuloma. The patient was considered a candidate for repeat surgical resection using a midline sub-occipital approach with the patient in the prone position to avoid the prior right far lateral craniotomy. A sub-occipital craniotomy, C1 laminectomy, midline durotomy, and subtonsillar approach were used. Neuromonitoring, SSEPs, and MEPs for cranial nerves 9, 10, 11, and 12 were utilized with the goal of maximal safe debulking using ultrasound, the operative microscope, and neuronavigation. Tissue samples were obtained for histological and immunohistochemical analyzes. The patient tolerated surgery well and was extubated. Postoperatively, she experienced bilateral numbness in all extremities with increased numbness on the right side. Baseline left-sided hemiparesis and bilateral hyper-reflexia remained. Muscle tone in the upper and lower extremities was increased without spasticity and tongue deviation was further to the right. Postoperative MRI demonstrated successful resection of the lesion [-]. Ultimately, the patient was ambulatory with assistance and transferred to the inpatient rehabilitation service on postoperative day 5. Pathological analysis revealed an epithelioid neoplasm with tumor cells displaying elevated mitotic activity with a Ki67 proliferation index of 19% on average and up to 26% in hotspots, distinct cell borders, and variable sheet-like, pseudopapillary, and trabecular architecture [-]. Multiple foci of bland necrosis with hyalinized vessels were also found, consistent with history of radiation treatment. Immunoreactivity for glial fibrillary acidic protein and epithelial membrane agent was both positive [ and ]. Tumor histopathology was consistent with recurrent astroblastoma and molecular classification allowed for further specific diagnosis as CNS HGNET with features of MN1-altered astroblastoma morphology. At 15-month follow-up, the patient had completed four cycles of bevacizumab and lomustine but continued to decline with worsening diffuse weakness and quadriplegia due to tumor recurrence. The patient required frequent assistance and medical care with a Karnofsky performance score of 50. Given her progressive decline with worsening weakness and quadriplegia, patient was referred to home hospice.