Intracranial arachnoid cysts are an uncommon, often developmental anomaly seen in both dogs and humans. Clinical signs, when present, are most often manifested as focal neurologic deficits or seizures. If clinical signs are present surgery is necessary for long term management. The two most common surgical approaches are cystoperitoneal shunting and cyst fenestration. In dogs and humans the debate as to which choice is preferred is ongoing. Data is limited in dogs, but thus far both surgical approached seem to carry a favorable prognosis. The aim of this paper is to provide an overview to the clinical presentation, etiology and treatment of intracranial arachnoid cysts.