This review is devoted to an urgent and insufficiently studied problem - the characteristics of the pubertal development of girls with cystic fibrosis (CF), the deviations in the period of menarche, the stages of the puberty, anatomical and physiological features of the female reproductive system in this monogenic disease. The increase in life expectancy, as well as the spread and increasing availability of targeted therapies, highlight the need for further research on this topic. Early diagnosis of disorders of pubertal development due to timely prescribed therapy (hormonal) allows not only correcting the timing of the onset of menarche in CF adolescent girls, but also increasing the chances of conceiving and bearing a child.