This case report describes the presentation, diagnostic evaluation, and management challenges encountered in an eight-month-old female infant with fever, seizure, and a large cystic brain lesion initially diagnosed as pilocytic astrocytoma but later demonstrating atypical teratoid/rhabdoid tumor (AT/RT) features on histopathological examination-the infant presented with a fever and cold persisting for 10 days, followed by a seizure episode. Laboratory investigations revealed abnormalities, including anemia and leukocytosis. Imaging studies identified a large cystic lesion causing hydrocephalus. Despite initial treatment, the patient continued to experience seizures, prompting surgical intervention. Debulking surgery was performed, resulting in postoperative motor deficits. Subsequent imaging revealed persistent lesions, leading to further surgical intervention with shunt placement. Histopathological examination confirmed pilocytic astrocytoma with features suggestive of AT/RT. Despite counseling regarding poor prognosis and recommendations for chemotherapy, the parents declined further treatment, and the patient was discharged. This case underscores the diagnostic complexity and therapeutic dilemmas associated with rare histological overlaps in pediatric brain tumors, emphasizing the importance of multidisciplinary collaboration and tailored treatment strategies for optimal patient care.