Autoimmune/lymphocytic hypophysitis is one of the rare causes of central diabetes insipidus in adults and is most common among women in the second or third trimester of pregnancy. Numerous studies have shown that lymphocytic hypophysitis is characterized by a very variable clinical signs with the development of neurological symptoms, visual disturbances and hypopituitarism with partial or complete loss of pituitary function, as well as a number of features in magnetic resonance imaging (MRI). Isolated lymphocytic indibuloneurohypophysitis occurs in fewer cases and involves the posterior lobe and stalk of the pituitary gland with a clinical presentation of diabetes insipidus. The above clinical case describes the development of hypophysitis in a pregnant woman with a predominant lesion of the posterior pituitary gland and an outcome in diabetes insipidus, which persists 6 years after pregnancy and childbirth. In the article some aspects of the differential diagnosis of diabetes insipidus in pregnant women, as well as instrumental diagnosis and treatment approaches of hypophysitis are discussed.