Hydrocephalus is a disorder characterized by increased accumulation of cerebrospinal fluid in the cerebral ventricles and/or subarachnoid space, leading to the dilation of these spaces. In the neonatal period, it occurs in 0,3 to 0,5 per 1000 live births. Based on the pathogenesis of hydrocephalus, it is classified into communicating and non-communicating types, depending on whether there is an obstruction in the flow of cerebrospinal fluid. The etiology of hydrocephalus is heterogeneous and classified into two major categories: congenital and acquired. Regardless of the etiology, the clinical presentation of hydrocephalus is similar in both cases. In the neonatal period, the predominant sign is macrocephaly, indicating an abnormally increased head circumference due to the open cranial sutures. Diagnosis is established based on the clinical presentation and neuroimaging techniques. The method of choice is brain ultrasound, which allows visualization of the ventricular system through the open anterior fontanelle without exposing the newborn to ionizing radiation. In some cases of congenital hydrocephalus, prenatal diagnosis is also possible. Therapeutic options are based on surgical methods of cerebrospinal fluid drainage. Drainage methods can be temporary or permanent. Temporary methods include external ventricular drainage, ventriculosubgaleal shunt, and ventricular reservoir, while permanent methods include ventriculoperitoneal drainage, ventriculoatrial drainage, ventriculopleural drainage, and endoscopic third ventriculostomy.