Complete androgen insensitivity syndrome (CAIS; also known as Morris syndrome) is a rare disorder associated with mutations in the androgen receptor gene located on the X chromosome. Individuals with CAIS, despite having a male karyotype, usually identify as female and exhibit female external genitalia. Management of CAIS patients requires a complex, interdisciplinary approach involving specialists in clinical genetics, gynecology, endocrinology, psychiatry and psychology. The aim of this study is to present the case of a 40-year-old patient diagnosed with an X chromosome mutation, including diagnostic procedures as well as pharmacological and surgical management.