Autosomal dominant polycystic kidney disease (ADPKD) is a common single gene hereditary disorder characterized by progressive development of bilateral renal cysts eventually leading to chronic kidney disease and an array of extrarenal abnormalities including intracranial aneurysm (ICAN). While the association of ICAN with ADPKD is undisputed, its management is not standardized. Conflicting data about the benefits of screening to the efficacy of different treatment modalities have not helped establish any opinion. We report three such cases managed surgically at Hiroshima University Hospital and discuss and review the literature regarding ADPKD and ICAN. Decision for surgical management of these cases were multifactorial and not exclusively because of ADPKD. There is a lack of sufficient randomized data on these topics and until such results are available, neurosurgeons are likely to resort to bit-part data and weigh the risk-benefit factor in individual patients and proceed accordingly.