Psychosis in epilepsy is mainly distinguished from the affective, schizophrenic, and cognitive domains by symptoms such as mood instability, anxiety, hallucinations and delusions, and confusion. Though its being rare, self-injurious or mutilating behavior is seen in seizure cases. In this report, we present an epileptic psychosis case with self-injurious behaviour. The patient was a 27-year-old primary school graduate, unemployed, single female, who had been admitted to our Psychiatry Clinic because of self injurious behaviors as well as visual and auditory hallucinations. Self-injurious behavior was reported to happen once a year for 3 years, suddenly begins, repeats the same word, and continues 1-2 hours. She carves up her arms with knife or a razor blade; she says a white-bearded man says he wants to do it. During such episodes, which lasted for one hour, she could not be inşuenced and she manifested restlessness. Furthermore she had mental and developmental retardation, retinitis pigmentosa, ventricular septal defect and hypogonadotropic hypogonadism. During the interview, she was speaking slowly. Her mood was low; affect was appropriate to her mood. Extensive neurological, somatic, and laboratory examination and magnetic resonance imaging of the brain, revealed no abnormalities. Electroencephalogram showed bilateral generalized sharp wave activity (left was more pronounced than right). The purpose of this report to discuss clinic aspect of psychosis in epilepsy with self-injurious behavior with the related literature and to emphasize that in all psychiatric patients, who were suspected for organic etiology, complete history, physical and neurological examinations and appropriate testing are essential for primary diagnosis. We also aimed to collect attention to a possibly new syndrome associated with other medical conditions accompanying epileptic psychosis.