Introduction: Turner syndrome is a genetic disorder that affects only females and is caused by the loss of the whole or a part of the X-chromosome. The aim of this study was to assess the quality of life and frequency of anxiety and depression in a cohort of Egyptian children with Turner syndrome (TS) diagnosed in Assiut university hospitals, Assiut, Egypt. Methods: In a case-control study; 40 females with Turner Syndrome (TS), aged 6-16 years, diagnosed in Assiut university hospitals, Upper Egypt, were compared to 40 age- matched apparent healthy girls as controls. They were subjected to full history taking, thorough clinical examination and assessment of pediatric quality of life; we used pediatric anxiety and depression scores. For cognitive assessment, health-related quality of life (HR-QOL) and psychological evaluation of anxiety and depression; we used the "Children Depression Scale" (CDI), "Children Anxiety Scale" Wechsler Intelligence Scale for children (WISC), and SF-36 by trained psychiatrists blind to the study. Results: Our study revealed Children Anxiety Scale scores ranged from 13-41 for patients with TS and 5-23 for controls. The mean anxiety scores were significantly higher in TS patients as compared to controls (p 0.001), (27.32 ± 7.1 versus 15.2± 2.9). In addition, we found CDI scores ranged from 38-70 for patients and 31-56 for controls. Mean depression scores were significantly higher in TS patients as compared to controls (p 0.001), (54.7 ± 8.8 versus 36.8 ± 9.9). The physical and mental component summary scores of HR-QOL were lower in TS patients when compared to controls. Conclusion: We concluded that female children with TS experience significant problems with HR-QOL, anxiety and depression, which need thorough psychiatric evaluation and rapid intervention of these patients.