Introduction: Amenorrhea and delayed puberty are rare manifestations of endocrinological disturbances in patients with chronic hydrocephalus. Fewer than 30 patients with these features have been reported to date. Case description: We present a case report of a 16-year old girl with primary amenorrhea caused by hydrocephalus due to congenital aqueductal stenosis. The patient presented with no other complaints. She had a normal height and slightly reduced sex characteristics for her age (P2, B3). Breast development commenced at age 11 but stalled at age 14. Blood tests revealed hypogonadotropic hypogonadism. Magnetic resonance images revealed marked noncommunicating supratentorial hydrocephalus without tumorous lesions but explained by aqueductal stenosis. The patient underwent endoscopic third ventriculostomy. Hormonology normalised and the patient experienced menarche only 3 years after surgery. Scoping review: Based on this case report, we conduct a systematic review according to the PRISMA guidelines for scoping reviews. This review focuses specifically on the diagnosis and treatment of aqueductal stenosis, which occurs at the junction of the third and fourth cerebral ventricles and leads to amenorrhea. Post-treatment outcomes will also be studied. Our aim is to determine the time between surgery and menstrual (re)occurrence. The review includes articles on the diagnosis of aqueductal stenosis -as a cause of amenorrhea-, as well as articles on treatment and outcomes.