Pregnancy and the postpartum period provoke an exacerbation of systemic lupus erythematosus (SLE), which requires aggressive cytostatic therapy that can cause life-threatening conditions for the fetus and complications for the mother, one of which is posterior reversible encephalopathy syndrome (PRES-syndrome). The article describes a clinical case of successful treatment of acute respiratory distress syndrome (ARDS) in a 22-year-old woman in labor with high activity of SLE during pregnancy complicated by the development of PRES-syndrome. Also, for the first time in the postpartum period, the patient was diagnosed with occlusive triventricular hydrocephalus, a giant cyst of the third ventricle. An emergency endoscopic cystoventriculocisternostomy was performed. In the future, the development of severe ARDS against the background of sepsis, which required the setting of extracorporeal membrane oxygenation (ECMO). A multidisciplinary approach and timely therapy saved the life of a young mother.