Genetic hydrocephalus is a neurological condition in which the cerebrospinal fluid (CSF) flows with subsequent and it results in enlargement of the cerebral ventricular cavities. The common cause of the congenital hydrocephalus is the variation in the L1CAM gene, and there is the narrow passage between the third and fourth ventricles. It is suggesting that hydrocephalus is more complicated than the simple CSF , and are many factors which are associated with the genetics of hydrocephalus, the major two factors are the i) telomeres proximity ii) and the more content of Adenine and Thymine [A, T] in the human CH as compared to the other nervous disorders. It is also suggesting that genetics of hydrocephalus is a crucial birth defect, and its genetics is still not completely understood, so it is the most important clinical feature. There are about 43 mutant loci associated with the animals and human hydrocephalus. Among them 9 are associated with animal models and 1 with the human. The most important hydrocephalus gene products are the growth factors, cytokines and many cellular signal pathways in the starting stage of brain development. In this study we will aim to understand the abnormalities in brain cause by the abnormal cellular functioning, and all these cellular events results congenital hydrocephalus. All these studies show that it is the mandolin type of disease with reference to the cellular, molecular genetics, physiological and pathological studies.