Objective: To explore the clinical symptoms, diagnosis and treatment of low intracranial pressure hydrocephalus (LPH). Methods: The ventriculo-peritoneal shunt (VPS) with pressure-adjustable valves was performed in 5 patients with LPH. The diagnosis of LPH in 2 patient was definitely made by lumbar puncture drainage before VPS. LPH developed in other 3 patients with normal pressure hydrocephalus during the following-up which the patients’ symptoms were not improved. Results: The following-up from 3 to 31 months after VPS showed that the clinical symptoms were improved and the enlarged ventricles significantly retracted in all patients. Conclusions: LPH is uncommon and occultly comes on. LPH is similar to normal pressure hydrocephalus in the clinical manifestation. CT and MRI show that there is ventriculomegaly in all the patients with LPH and periventricular edema in some patients with LPH. VPS with pressure-adjustable valve is an effective method to treat LPH. The perfect threshold value of pressure adjustable valves, which is suitable for the individual, may be got by more than once adjustment and long-term observation of the curative effect.