**1. Introduction**

Arterial hypertension, affecting about one billion people worldwide, is the most prevalent modifiable risk factor for cardiovascular diseases and related disability [1]. Essential hypertension is a multifactorial condition, resulting from a complex interaction between lifestyle and genetic factors. A positive family history increases the overall risk of developing high blood pressure and genetic factors account for 30–50% of the individual risk [2]. A minority of the hypertensive patients are affected by an inherited disease, resulting from single gene germline mutations affecting mineralocorticoid, glucocorticoid or sympathetic pathways [2,3]. Among these diseases, Liddle syndrome (LS) is caused by point mutations of the epithelial sodium channel (ENaC), that cause renal aldosterone-independent sodium reabsorption. The aim of this review is to provide an update on the current knowledge of LS, including the genetic and pathophysiological basis, the clinical features, the diagnostic and medical management and a new case is reported.
