Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm and that usually arises in the posterior fossa, survival from this is frequently poor. We present a unique case in a 21-month-old girl who had an atypical teratoid/rhabdoid tumor with cystic components located in the right fronto-parietal lobe. The patient underwent radical surgical intervention followed by chemotherapy. It consisted of five chemotherapeutic agents, but the patient did not receive any radiotherapy. The postoperative course was uneventful and the patient was followed-up by cranial magnetic resonance imaging every 3 months. Two years later at the last follow-up visit, there was no evidence of a tumor relapse on MRI, and the examination was symptom free. It is possible the favorable outcome of the patient resulted from a rapid diagnosis, prompt management, radical surgical intervention and aggressive chemotherapy.