Up to date less than 300 observations of Itsenko-Cushing’s syndrome in pregnant women have been published in the literature, which makes the diagnosis and treatment of an already severe disease extremely difficult and does not allow the development of clear clinical recommendations for the management of such patients. The primary cause of Itsenko-Cushing’s syndrome during pregnancy is adrenal corticosteroma, less often Itsenko-Cushing’s syndrome is detected, induced by chorionic human gonadotropin and hormonally active adrenocortical carcinoma, the latter has an unfavorable prognosis. High clinical alertness is important for rapid diagnosis and timely treatment, which significantly reduces the incidence and severity of complications in the mother and fetus and improves pregnancy outcomes. Diagnosis should be based on specific signs and symptoms: cushingoid body type, striae, ecchymosis, acne and hirsutism, impaired carbohydrate metabolism and arterial hypertension. If suspected, the most preferable are studies of cortisol in daily urine and evening saliva, detection of a violation of the circadian rhythm of cortisol secretion, as well as a blood test for basal ACTH. Ultrasound and magnetic resonance imaging without contrast enhancement can be used as imaging modalities. It is optimal to perform laparoscopic adrenalectomy of the tumor in the second trimester of pregnancy, followed by therapy for adrenal insufficiency. In the diagnosis and effective treatment of patients with Itsenko-Cushing’s syndrome before pregnancy, the use of non-hormonal contraceptives is recommended until a stable remission of the disease is achieved.