Abstract: Objective To improve the cognition of the disease by exploring the clinical feature,diagnosis and therapeutic method of Atypical teratoid/rhabdoid tumors (AT/RT).Method Clinical data of one patient diagnosed AT/RT by postoperative pathology was studied retrospectively,domestic and foreign related reports and studies of such disease were reviewed.Results The symptom of severe headache was obviously alleviated after surgical treatment,the patient followed- up abandoned postoperative radiation and chemotherapy and died after 5 months postoperatively.Conclusions AT/RT is a rare disease with poor prognosis,high recurrence and mortality.Postoperative adjuvant chemotherapy and radiation can improve the prognosis of patients.