Patients with autosomal dominant polycystic kidney disease (ADPKD) have a higher incidence of intracranial aneurysms (ICA) than the general population. These ICA also rupture at an earlier age in patients with ADPKD and are associated with high morbidity and mortality. In a recent study, 25% of patients with ADPKD with a documented ICA demonstrated a new ICA on follow-up. It is not known, however, whether patients with ADPKD who have had a negative ICA imaging study would demonstrate an ICA on a repeat imaging study. Only 2 (2.6%) of 76 patients with ADPKD with an initially negative study demonstrated an ICA on follow-up, despite the high frequency of risk factors such as hypertension, smoking, and a family history of ruptured ICA. The mean length of follow-up was 9.8 yr (median, 9.7 yr). These findings have important health care and economic implications in following patients with ADPKD.