Disorder of the formation of the cavum septum pellucidum (CSP) occurs in a wide range of pathologies of the brain. Its identification from 18 to 37 weeks is a necessary rule of prenatal screening, which must also be performed in postnatal studies of premature infants. Unimaged CSP should be considered as a potential indicator of cerebral dysfunction and such severe malformations as agenesis (dysgenesis) of the corpus callosum, holoprosencephaly (HPE), schizencephaly, Aicardi syndrome, hydrocephalus and septo-optic dysplasia (SOD). A special place in the differential diagnosis of unimaged CSP is represented by SOD, lobar HPE, and its mild subtype - septopreoptic HPE. The literature describes the isolated absence of SP, without finding other signs related to HPE or SOD, as a variant of development. However, the neurological outcome in these children varies from normal to delayed development of the nervous system by different degrees, so this statement remains controversial. The article discusses in detail the anatomy, physiology, pathology of the septal region, the role of echographic studies in the antenatal and postnatal periods, and the correlation of the detected changes with the clinical picture.