Aquaporins (AQPs) are membrane proteins that facilitate water and small solute movement in tissues. Hydrocephalus is the major central nervous system disorder associated with defective cerebrospinal fluid turnover. Aquaporin-4 (AQP4) is a water channel located mainly at the blood-brain barrier (BBB) and blood-cerebrospinal fluid (CSF) interfaces and is associated with the elimination of cerebral edema via these routes. The aim of this study is to review the pertinent literature concerning the role of AQP4 in the pathophysiology of hydrocephalus.We performed a MEDLINE search using the terms aquaporin AND hydrocephalus. The results of the search were further refined to exclude studies not related to aquaporin-4.Six studies were identified. All studies utilized an animal model such as AQP4-knockout mice, H-Tx rats, and kaolin and L-α-lysophosphatidylcholine (LPC) stearoyl injection models of hydrocephalus. Most studies indicate that there is an up-regulation of AQP4 expression at the BBB and blood-CSF interfaces in cases of hydrocephalus. One study, reported sporadic cases of obstructive hydrocephalus in a subgroup of AQP4-knockout mice.Few publications have studied the association between aquaporins and hydrocephalus. Currently, all the existing studies rely on animal models. An adaptive and protective role of AQP4 to increase the resolution of the "hydrocephalic" edema at the BBB and blood-CSF interfaces is proposed in the pathophysiology of hydrocephalus. Further research is needed to clarify if this association exists in humans.