Background/Objectives: Holocord astrocytomas are exceptionally rare intramedullary tumors, especially in adults, and often present with extensive longitudinal growth. Because only a small number of cases have been described, management strategies remain insufficiently defined. This report presents an adult patient treated with a staged surgical approach and provides an updated review of the literature. Methods: A 31-year-old male presented with progressive paraparesis, sensory deficits, and sphincter dysfunction. MRI demonstrated an intramedullary tumor extending from T3 to the conus medullaris. The patient underwent a planned two-stage resection with intraoperative neurophysiological monitoring. Histopathological and DNA-methylation analyses were performed. Additionally, a systematic review of previously reported holocord astrocytoma cases was conducted. Results: The two-stage surgical strategy enabled extensive debulking across multiple spinal segments while preserving neurological function. The patient demonstrated marked postoperative improvement, including restoration of sphincter control, improved motor function, and better mobility. Histopathological analyses confirmed a high-grade astrocytoma with piloid features. The literature review identified 28 previously reported cases, including only 5 in adults. Reported neurological outcomes across adult cases are variable, reflecting the heterogeneity and rarity of this tumor entity. Conclusions: Holocord astrocytomas in adults are extremely rare and pose particular diagnostic and therapeutic challenges. This case demonstrates that a carefully planned, staged surgical approach can achieve meaningful neurological recovery, even in patients presenting with severe preoperative deficits. The report expands the limited body of evidence available for adult holocord astrocytomas and may support future management strategies.