We report a case of long-standing overt ventriculomegaly without aqueductal stenosis. A 66-year-old woman developed progressive gait disturbance. Her gait was wide-based, with a difficulty in turning. Prominent macrocephaly was present, which suggests the presence of long-standing hydrocephalus since childhood. Cognitive impairments were detected on neuropsychological tests. Cerebral magnetic resonance imaging revealed marked enlargement of the third and lateral ventricles. Aqueductal stenosis was not apparent; however, endoscopic third ventriculostomy partly ameliorated gait disturbance. Endoscopic third ventriculostomy can be a therapeutic option for long-standing overt ventriculomegaly even if the aqueduct is patent.