IMPORTANCE: Autosomal dominant polycystic kidney disease (ADPKD) has been associated with cardiovascular abnormalities such as intracranial and aortic aneurysms.OBJECTIVE: To systematically review the case reports and case series of ADPKD patients with coronary artery dissection or aneurysm. Evidence review Systematic review registration number: CRD42015015723.DATA SOURCES: MEDLINE, Web of Science and OpenGrey, reference lists of studies.STUDY SELECTION: Published case reports and case series.DATA EXTRACTION: Two parties analyzed the studies. Disagreements were solved by consensus or by a third party.FUNDING: none. Findings The reports of 23 patients (22 from 17 studies--six with coronary artery dissection and 16 with coronary artery aneurysm--and one with coronary dissection) were analyzed and reported here. Most patients were symptomatic. Coronary dissection showed female and left descending anterior artery predominance, features similar to non-ADPKD patients, but a median diagnostic age below expected (41 vs. 50 years old). Coronary aneurysms had male and right coronary artery predominance but lower median diagnostic age (44 years old) and higher rate of multiple vessel affection than reported for non-ADPKD patients.CONCLUSION AND RELEVANCE: Clinical disparities may suggest a different mechanism of aneurysm formation compared to the population without ADPKD. Nevertheless, lack of access to data of one patient and text of one article limited our conclusions. Coronary aneurysms and dissections represent a source of coronary syndromes and death in ADPKD. Mutation of ADPKD-related genes may predispose to coronary abnormalities, especially aneurysms. Further analysis regarding this association is necessary.