Monogenic forms of low renin hypertension can now be identified in a large and heterogeneous family of hypertensive patients with highly specific etiologies and similar clinical manifestations. These include the following well-characterized disorders: apparent mineralocorticoid excess, Liddle's Syndrome, steroid 11beta-hydroxylase (11beta-OHD) and steroid 17-hydroxylase (17-OHD) deficiencies, glucocorticoid-remediable hyperaldosteronism (familial hyperaldosteronism type I), familial hyperaldosteronism type II, hypertension exacerbated by pregnancy and primary hyperaldosteronism (Conn's syndrome). The successful elucidation of specific DNA mutations in most of these conditions has emphasized the role of molecular genetics in hypertension, a field in which diagnosis can now be made on proven genetic evidence. The current knowledge of these genetic markers enables practitioners to make precise diagnoses, and to initiate specific therapy, in patients with these relatively uncommon but interesting and often treatable forms of hypertension.