Hydrocephalus is a congenital or acquired neurological disorder in dogs, associated with the excessive accumulation of cerebrospinal fluid (CSF). The factors contributing to hydrocephalus development are diverse and not always possible to determine. The aim of this article was to present two cases of the diagnosis and treatment of internal hydrocephalus in dogs. The observations included animals with neurological disorders of unknown origin. Internal hydrocephalus was diagnosed based on the results of magnetic resonance imaging, and the treatment of both patients comprised ventricular brain drainage. The surgical treatment was fully effective for one dog. For the other dog, a short-term improvement was followed by deterioration in condition due to occlusion of the ventriculoperitoneal drain, and the owners decided to euthanize the animal. The analysis of our cases led to the conclusion that congenital hydrocephalus is a disorder that challenges veterinarians in terms of diagnosis and therapy, due to limited access to advanced imaging techniques such as MRI or CT, and from the complicated nature of the surgical treatment. However, it should be noted that if the disorder is correctly diagnosed and ventricular brain drainage is adequate, the chances for successful functioning are significant.