Background: Overdrainage and collapse of the hemispheres is a potential severe complication after surgical treatment of internal hydrocephalus using ventriculoperitoneal shunts. Here we describe a case of a spontaneous hemispheric ventricular collapse in an untreated dog with congenital hydrocephalus internus. Case presentation: A twelve-week-old, male, intact Golden Retriever was presented with a history of peracute obtundation, impaired vision, and progressive gait abnormalities of all limbs for three days. Neurological examination revealed a dome shaped skull, a broad-based stance and a moderate cerebellar ataxia. The postural responses were markedly delayed in all limbs. Moderate ventro-lateral strabismus, vertical nystagmus and absent menace response were observed bilaterally. Clinical signs indicated multifocal localisation (forebrain, cerebellum). Magnetic resonance imaging (MRI) showed dilation of all cerebral ventricles, irregular thinning of the periventricular white and grey matter, consistent with internal hydrocephalus. In addition, the hemispheres were collapsed at the right temporal and left frontal lobe with haemorrhage filling the adjacent subarachnoid space. The dog underwent left frontal and right temporal craniotomy for removal of the haemorrhage. The dog improved on all neurological signs and was discharged after seven days. A repeat MRI three months postsurgical intervention showed reexpansion of the cerebral hemispheres. Subarachnoid haemorrhages were markedly reduced. Conclusions: Collapse of the hemispheres can occur spontaneously in dogs with hydrocephalus internus. Removal of the haemorrhage can improve clinical signs.