The article presents a review of the literature on the state of the problem of diagnosis and treatment of congenital central nervous system (CNS) tumors in children. Oncological alertness and awareness of specialists supervising pregnant women, parturient women, and young children, diagnostic features and the level of technological assistance to children with congenital CNS tumors determine the survival and prognosis of the disease. Modern methods of diagnosis and treatment make it possible to achieve satisfactory results in this category of patients. In the management team of patients and families, it is necessary to involve a palliative specialist, especially in cases of detection of noncurable tumors. Own clinical observations of patients with congenital CNS tumors are presented.