Arterial hypertension is a severe public health problem. Due to a sedentary lifestyle and the obesity epidemic, the prevalence of primary arterial hypertension increases every year. However, the presence of secondary hypertension should not be overlooked. One of the forms of secondary hypertension is monogenic hypertension, a single gene-dependent condition associated with moderate to severe drug-resistant hypertension and a higher risk of developing neurological and cardiovascular complications. It should be considered in patients with coexistence water and electrolytes imbalance or rarer, with precocious or delayed puberty, growth deficiency, and brachydactyly. Characteristic features for most monogenic hypertension cases comprise impairment of water and electrolyte balance, most often hypokalemia and metabolic alkalosis. Identifying patients with monogenic hypertension is of utmost importance to implement appropriate treatment and reduce the risk of complications.