Autosomal dominant polycystic kidney disease (ADPKD) belongs to the most common genetic disorders, and affects approximately 1 in 1,000 people in the general population. ADPKD is a systemic disease, with multiple extrarenal manifestations, like arterial hypertension, aneurysms, and cysts in solid organs, including liver, pancreas, and spleen. Among intracranial manifestations of ADPKD, the most important are intracranial aneurysms. ADPKD is a well-known risk factor for them. Additionally, arachnoid cysts may be found in some ADPKD patients. Rarely, pineal cysts and choroid plexus cysts are observed in this population. The aim of our paper is to present intracranial manifestations that may be observed in ADPKD.