An atretic cephalocele is a rare birth defect in which a small sac-like or nodular mass forms on the surface of the skull in the suture or midline area. It is a remnant of the original herniation of the meninges and sometimes brain tissue through a defect in the skull, which subsequently closes and scars. The article describes the typical appearance of such a formation on the head of a newborn, as well as its imaging findings. Imaging methods, especially magnetic resonance, show the connection of the formation with intracranial structures or small defects in the skull. Atretic cephalocele is often associated with other anomalies of brain development or cerebral vessels, which is essential for further examination of the child. The diagnosis is established by a combination of physical examination and imaging methods. Treatment is usually surgical, especially if there is a risk of injury, infection or accompanying complications are present.