We studied the development of congenital hydrocephalus found in a colony of an inbred strain of Wistar-Lewis rats (LEW/Jms) at various intervals after birth. The disorder was transmitted as a simple recessive mendelian character. Hydrocephalic neonates were recognized 2 days after birth by stretching of the skin over the head. Death usually occurred between 10 and 20 days of age. The findings suggested the possibility of a disturbance of cerebrospinal fluid circulation resulting from primary occlusion of the 3rd or lateral ventricles during embryological development. In later phases, the hydrocephalus was aggravated by obliteration of the subarachnoid space and by stenosis of the aqueduct occurring secondary to compression of these structures from increased pressure within the brain. In some animals, external hydrocephalus occurred as a result of rupture of the occipital pole and the establishment of a direct communication between the lateral ventricles and the subdural space. We looked for antibodies against viruses that have been known to produce hydrocephalus in experimental animals and obtained entirely negative results.