Disorders of sex development (DSD) are rare, but represent one of the most difficult diagnostic and therapeutic problems. Until now it has not been established when surgical interventions in patients with DSD should be undertaken.Androgen insensitivity syndrome (AIS, Morris syndrome) is a congenital, inherited in a recessive, X-linked manner disorder of sex development. The prevalence of the disease is estimated for 1: 20,000 to 1:64,200. According to the severity of androgen resistance AIS can be classified into three categories: complete androgen insensitivity syndrome (CAIS), partial androgen insensitivity syndrome (PAIS) and mild androgen insensitivity syndrome (MAIS). Diagnosis of AIS requires clinical, hormonal and molecular investigation.We present a case of 17-year-old girl referred due to primary amenorrhea. Her cytogenetic analysis revealed the kariotype of 46, XY. Hormonal and imaging investigations confirmed the diagnosis of complete androgen insensitivity syndrome.