Androgen insensitivity syndrome (AIS) is an inherited disorder of sexual development caused by mutations in the androgen receptor encoding gene. A female patient at the age of 17, was diagnosed with complete androgen insensitivity syndrome, during the diagnosis of primary amenorrhea. She was assigned grade 7 on the Quigley scale. Cytogenetic analysis showed a 46 XY karyotype. A gynecological examination revealed a blind-ending vagina and a lack of a uterus. Physical examination revealed normal breast development and scanty pubic and axillary hair. At the age of 18, the patient underwent laparoscopic gonadectomy. Before the surgery, the testosterone level was 5.6 ng/ml, and after 0.6 ng/ml. Histopathologic examination of the gonads revealed a cyst and many lumps and in one of them, a tubular adenoma was diagnosed. After the procedure, the patient was under medical supervision and was taking orally 1 mg of estradiol daily. At the age of 24 patient was diagnosed with osteoporosis, T-SCORE was -2.6 SD, and 3 years later -2.76 SD. The patient received sodium alendronate and ibandronic acid. At the age of 34, she was diagnosed with type 2 diabetes, therefore she started receiving metformin and was under diabetological supervision. Quigley grades 6 and 7 correspond to complete androgen insensitivity. The genitals are completely feminized, and newborns at birth are assigned as females. The diagnosis is usually established during the diagnostics of primary amenorrhea. CAIS is associated with high risks of gonad carcinogenesis and therefore a gonadectomy must be performed. Hormone replacement therapy (HRT) is required after gonadectomy to maintain secondary sexual characteristics. Due to the high risk of osteoporosis, the patient requires calcium and vitamin D supplementation and bone density control. The prognosis is good, and gonadectomy and HRT usually give satisfactory long-term results.