The World Health Organization estimates that worldwide about one million pregnant women are adversely affected by infection with Treponema pallidum. It results in abortions, stillbirths, congenital syphilis or premature, low-birth-weight babies. The majority of cases of congenital syphilis are seen in developing countries, but the disease is unexpectedly frequent in high-income populations. Usually, neonates do not have specific symptoms of the disease. The most relevant issues concerning congenital syphilis are presented in the paper: the necessity and the principles of screening programmes in pregnancy as the best prevention tool, treatment recommendations for infected pregnant females, as well as new methods and modified guidelines for the diagnostics of congenital syphilis.