Turner Syndrome is a genetic syndrome in which a woman lacks one X chromosome, giving a karyotype of 45XO, or, alternately, a mosaic genotype with both 45XO and normal 46XX cells. Physical features include short stature, failure or reduction in secondary sex characteristics development, gonadal dysgenesis, and superficial features including neck webbing, hair loss, and cubitus algus. Women with turner syndrome (TS) are of generally normal intelligence, but may have some specific defects in spatial orientation. Psychiatric condition has not been defined traditionally to TS, and TS is not mentioned in DSM-IV. Studies about the psychological aspects of TS have focused on the inşuence of the physical stigma of TS on psychological development in young female population, highlighting short stature, failure in sexual maturation compared to age peers, the issue of infertility, and how these issues relate to self-image and femininity. A “TS personality” characterized by immaturity, depressiveness, passivity, distractibility, and docility is suggested, although no rigorous scientific study has confirmed these claims. Psychosis is relatively rare, but some authors have hypothesized that psychosis could be associated with TS. We present here a case of 20 years old, single woman, who was diagnosed TS, and had psychosis. At the age of 19, she had social withdrawal, insomnia, decreased self care and auditory and visual hallucinations, delusions of persecution. After risperidone 1 mg treatment, extrapyramidal symptoms began. So her treatment changed to olanzapine, olanzapine dose was increased up to 20 mg. However, extrapyramidal symptoms began and psychotic symptoms didn’t decrease. Therefore olanzapine was stopped, and clozapine was started, clozapine dose was increased up to 100 mg. Extrapyramidal symptoms recovered, auditory and visual hallucinations, delusions of persecution decreased, communication with people was normalized, she returned to premorbid functionality. TS is a unique syndrome; it is important to identify it in order to avoid labeling patients with a more severe diagnosis, to determine its frequency and epidemiology, to understand its biological basis, and most importantly, to identify optimal treatment strategies. The majority of TS patients are of normal intelligence, social functioning and employment, yet the case reports of psychiatric disorders in this syndrome are similar and were considered unique enough to warrant description. Despite the descriptive information, the literature lacks rigorous, statistical analysis directed toward identifying the described syndrome in TS. Our case is about the treatment of a patient, who has both TS and psychosis. TS patients may have different sensitivity to antipsychotics, as observed with our patient. She had extrapyramidal symptoms with low dose risperidone and olanzapine, which we don’t expect in common. This case report suggests that the use of clozapine in TS following the recommended precautions could be safe and effective.