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Pregnancy in patients with an advanced stage of chronic kidney disease (CKD) remains a rather rare situation to date. This observation demonstrates our own experience of successfully management of pregnancy in a patient with chronic kidney disease stage 4. A special feature of this observation is an unclear diagnosis that led to CKD. Based on a combination of advanced CKD in a young patient with no kidney history, no changes in urine tests, increased blood pressure, hyperuricemia, and small cysts of both kidneys, a diagnosis of autosomal dominant tubulo-interstitial kidney disease was suggested, despite the lack of family history of renal disease. Since the kidney disease was first identified during pregnancy, the main areas of care were the correction of complications (anemia, calcium-phosphorus disorders), caused by the advanced stage of CKD and the prevention of pre-eclampsia as one of the most frequent complications of pregnancy in this cohort of patients. In order to timely diagnose preeclampsia, the patient was regularly monitored for angiogenesis markers. Conducting pregnancy was carried out by an interdisciplinary team of specialists (nephrologists, obstetrician-gynecologists). Pregnancy ended with the birth of healthy baby. After childbirth renal failure progressed.