In the new classification of disorders of sex development (DSD) Morris syndrome is included in the group of 46,XY DSD. It is determined by the insensitivity to androgen receptors, its prevalence being 1:20,000–1:100,000 from genetic males. The patients have male karyotype and testes, but their organs, tissues are insensible to testosterone, therefore a female phenotype develops with breast development, lack of pubic and axillary hair (“hairless women”). The 48 years-old patient (P.M.) was operated for bilateral inguino-labial hernias, and the gonads detected in the hernial sac – considered as ovaries – were replaced in the abdominal cavity. She addressed to endocrinologist for primary amenorrhoea and infertility. She has blind vaginal pouch, lack of uterus and adnexa, breast development and lack of androgen-dependent hair. The testosterone-level, normal previously, decreased considerably at 37 years of age, after extirpation of testes.
Keywords: disorders of sex development, complete androgen insensitivity syndrome