ObjectiveTo review the diagnosis, clinical characteristics and treatment of Arachnoid cyst rupture associated with epilepsy. MethodsThe clinical data of one patient with arachnoid cyst rupture associated with epilepsy was reported and diagnosis, clinical characteristics, the treatment options were discussed with literature reviewed. ResultsWe arranged the operation:arachnoid cyst resection and the left anterior temporal lobe resection and colostomy, the patient recovered well postoperatively, without special discomfort, epilepsy did not attack again. ConclusionsArachnoid cyst rupture associated with epilepsy is extremely rare, postoperative effect is good through strict preoperative assessment.