Congenital hydrocephalus results from diverse causes which could be developmental, genetic, infective and haemorrhagic in nature; aqueductal stenosis being the most common of all. Altered fluid dynamics results in loss of brain substance and enlargement of CSF spaces with the resultant sequelae. The present article reviews the multifaceted problems of congenital hydrocephalus from the standpoint of its pathophysiology, clinical ramifications & treatment.