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Atypical teratoid/rhabdoid tumor (ATRT) is a rare and highly malignant central nervous system (CNS) neoplasm, most commonly diagnosed in children and only infrequently reported in older adults. Classified as a World Health Organization (WHO) grade IV tumor, ATRT carries a poor prognosis and typically requires aggressive, multimodal treatment. Management in elderly patients is particularly challenging due to comorbidities, a lack of evidence-based guidelines, and heightened risk of treatment-related toxicity. We report the case of a 75-year-old woman with hypertension and hyperlipidemia who presented with progressive dizziness, headache, and blurry vision. Imaging revealed a pituitary macroadenoma compressing the optic chiasm. Following transsphenoidal hypophysectomy, histopathology unexpectedly confirmed ATRT, CNS WHO grade IV. The patient initiated craniospinal radiation and systemic chemotherapy; however, her course was complicated by severe thrombocytopenia, critical illness myopathy, and persistent encephalopathy attributed to prolonged steroid use and metabolic derangements. Interval imaging demonstrated stable disease with reduced mass effect on the optic chiasm. Due to poor treatment tolerance, radiation therapy was discontinued after 26 sessions, and she was discharged to rehabilitation with plans for continued chemotherapy. Unfortunately, her condition further deteriorated due to treatment-related complications, and she ultimately passed away under hospice care. This case underscores the diagnostic and therapeutic challenges of ATRT in older adults, a population for whom standard pediatric-based regimens may be excessively toxic. It highlights the need for individualized, patient-centered treatment strategies that prioritize disease control, treatment tolerance, functional status, and quality of life.