Autosomal Polycystic Kidney Disease ( ADPKD) is the most common inherited renal disease. ADPKD is caused by mutations in PKD1 and PKD2, encoding polycystin 1 and 2, respectively. ADPKD is a systemic disease, with renal and extrarenal involvement. Renal disease is characterized by formation and growth of cysts, with progressive destruction of renal parenchyma and development of End Stage Renal Disease (ESRD) in about 50% of affected individuals at the age of 60 years. Extrarenal disease usually involves the liver, heart and vasculature. Cardiovascular manifestations occur in a high percentage of patients with ADPKD, including hypertension, left ventricular hypertrophy, cardiac valvular abnormalities, and intracranial aneurysms. An early treatment of hypertension may decreased the risk of cardiovascular complications, the leading cause of morbidity and mortality. The antihypertensive agents of choice should be ACE inhibitors and angiotensin II receptor antagonists. In this review, we will focuses on the cardiovascular problems of patients with ADPKD.