Introduction: Although rare, chronic hydrocephalus may cause amenorrhea and delayed puberty associated with obesity as the main endocrine manifestations. Since the first operation carried out in 1950 on a patient with amenorrhea and hydrocephalus, fewer than 30 patients with these features have been reported. Case Report: Our patient represents a teenage case of primary amenorrhea, partial adrenal insufficiency and adult growth hormone deficiency caused by hydrocephalus due to aqueductal stenosis. Our English-language literature search for similar well-documented cases yielded less than 30 cases. Our management consisted of endoscopic third ventriculostomy, which resulted in regular menstrual cycles and normalization of somato- and adrenocorticotropic axes. Discussion: This is a rare case of a 20-year-old woman with primary amenorrhea caused by hydrocephalus due to aqueductal stenosis. Neurosurgical treatment produced relief in hypothalamic hydrodynamics tension, allowing recovery of hormonal function and restoring normal menstrual cycles.