This study aimed to evaluate the anxiety, depression and quality of life scores of children and adolescents diagnosed with familial Mediterranean fever (FMF) and compare these scores with the ones of healthy controls. The study group comprised of 130 children and adolescents diagnosed with FMF with a mean age of 12.6 +/- 2.58 and control group comprised of 121 healthy controls with a mean age of 11.8 +/- 2.84. Both groups were evaluated with child depression inventory (CDI), screen for child anxiety related emotional disorders (SCARED), pediatric quality of life inventory TM 4.0 (PedsQL (TM) 4.0) questionnaires. The severity of FMF was assessed by Pras scoring system as mild, moderate and severe disease. The comparisons of these three subgroups of FMF were made in terms of anxiety, depression and quality of life. Children and adolescents with FMF had significantly higher median scores of anxiety than healthy controls. The median scores of depression and quality of life were similar between both groups. Patients with a depression score of 19 or above had significantly higher scores of anxiety and longer duration of disease than the patients with a depression score below 19. While the patients with a severe course of FMF had higher median scores of depression and anxiety, they had significantly lower median scores of quality of life. According to our evaluation, patients with FMF had higher anxiety scores and as the disease become severe, not only anxiety scores but also features of depression become overt. An early apprehension of the mood changes of these patients may have a positive influence in the management of FMF. So, a close collaboration between child and adolescent psychiatrist and pediatric rheumatologist is essential for all over well-being of children and adolescents with FMF.