Background: Congenital hydrocephalus (CH) is a complicated syndrome that may be linked to a wide range of brain disabilities. It results from restricted cerebrospinal fluid flow either functionally or physically, causing advanced cerebral ventricular dilation and compression of brain tissue. The physical characteristics of CH are persistent open anterior fontanelle, dome shape head, and ventrolateral strabismus on both sides. There are also, headache, nausea, crossing normal centile lines, sunset eyes, distended scalp veins, delayed development, poor feeding, as well as parinaud's syndrome and possible abducent nerve palsy. CH is linked to variable risk factors including prematurity, congenital infections, maternal pathologies, medications, alcohol use, trauma and tumors. It may be related to other congenital abnormalities like spina bifida, aqueductal stenosis, Arnold-Chiari and Dandy-Walker anomalies. This review summarizes CH as regards the pathophysiology, risk factors, genetics, clinical picture, and associated CNS defects, providing information that can help in prevention of some cases of CH. Conclusion: Protection of infants against CH could be achieved by avoiding risk factors, early diagnosis and proper management