Congenital factor V deficiency (FVD) is a rare bleeding disorder due to an inherited mutation. So far, there are no standard protocols for pre- and peri-operative management of patients with factor V deficiency. This poses a challenge for surgeons and requires a multidisciplinary approach. We present a case of a 60-year-old woman with factor V deficiency admitted to the neurosurgery department of Ibn Tofail Hospital for hydrocephalus requiring a ventriculoperitoneal shunt. Pre-operative management of the patients as well as outcome and follow-up are described and compared with relevant literature.