An overview of new insights in pathogenesis of intracranial manifestations-especially concerning intracranial aneurysms (ICA) and subarachnoid hemorrhage-in autosomal dominant polycystic kidney disease (ADPKD) is given. Results of CT examinations of 65 patients with ADPKD are presented: they disclosed findings suspects of intracranial aneurysms in 4 patients, other vessel abnormalities and malformations in 6 patients and cystic dilatations in cisterna magna-of arachnoid cyst type in 6 patients. A case story of a patient successfully treated for subarachnoid hemorrhage due to aneurysm rupture is presented. Indications and screening methods in ADPKD patients with ICA are discussed. Brain abnormalities in ADPKD-especially ICA-suggest the complex nature of this genetic disorder, and regarding to the possible clinical impact, should be adequately managed.