The authors report 14 cases of unilateral hydrocephalus in adults. Headache was the most common presenting symptom. Unilateral hydrocephalus was documented in each patient with computed tomography scans; magnetic resonance imaging was also used in seven patients in the latter part of the series. Unilateral hydrocephalus was caused by tumor (seven patients), venous angioma (one patient), ependymal cyst (one patient), postinflammatory gliosis (one patient), and was idiopathic in four patients. The primary surgical treatment was craniotomy with fenestration of the septum pellucidum, which relieved symptoms in eight of nine patients for whom long-term follow-up data were available.