Autosomal dominant polycystic kidney disease (ADPKD) is an inherited kidney disease which leads to progressive kidney failure. About 5–10% of patients requiring renal replacement therapy are affected by ADPKD. Cardiovascular diseases are the main causes of morbidity and mortality in these patients with ADPKD; arterial hypertension (AH) is the first symptom with a very early onset. Anyway, some other cardiovascular abnormalities have been reported in ADPKD regardless of the presence of AH. With this background, we conducted a systematic review, collecting all randomized controlled trials (RCTs) and quasi-RCTs found on the main databases; we evaluated the evidence about different imaging techniques to grade the cardiovascular risk in a very early stage of disease. This review aims to describe all cardiovascular assessments in ADPKD patients to improve clinicians’ ability to discover cardiovascular involvement early, allowing appropriate therapies promptly.