Congenital adrenal hyperplasia (CAH) is a an autosomal recessive disorder resulting from a defect in any of several enzymes involved in biosenthesis of cortisol and sex steroids in adrenal cortex. 21-hydroxylase deficiency (90-95%) is the most common form and 17 alpha-hydroxylase / 17,20 lyase deficiency is the least common (<1%). These enzyme deficiencies result in inadequate cortisol production and consequently increased production of corticotropin-releasing hormone (CRH) and adrenocorticotropin hormone (ACTH). Glucocorticoids and sex steroids are used for this hormonal production disorder as a long time treatment. Therefore, anesthesia management is characteristic in this patient group. We aimed to present general anesthesia management for a thyroidectomy operation planned for a 38-year-old female patient with 17 alpha-hydroxylase / 17,20 lyase deficiency with a history of steroid use.