Summary: Renovascular hypertension (RVHT), usually caused by renal artery stenosis (RAS), is one of the most common causes of secondary hypertension. RAS may result in the development of resistant hypertension, a progressive decline in renal function and cardiac destabilization syndromes, which may include flash pulmonary edema or congestive heart failure.The most common cause of RVHT is atherosclerosis of the main renal artery, and most of the remaining cases are attributed to fibromuscular dysplasia (FMD). Less commonly encountered types of renal artery disease associated with RVHT include renal artery aneurysm (RAA), arteriovenous fistula and other causes. Of note, the existing literaturę dealing with these rare entities mostly consists of case reports, small clinical series, or reviews. In addition, the prevalence and incidence of these lesions and malformations in the general population is not clearly established and may be overestimated, as most cases have been diagnose in tertiary referral centers. In recent decades, such rare causes of RVHT are, nevertheless, increasingly identified due to extensive use of noninvasive imaging techniques.