Summary: Primary aldosteronism is considered the most common form of hormonal hypertension. Sporadic PA constitutes the vast majority of patients; however, familial hyperaldosteronism occurs even in up to 6% of cases. Type I of familial hyperaldosteronism is caused by the presence of chimeric CYP11beta1/beta2 gene, which is controlled by adrenocorticotropic hormone. Other types of familial hyperaldosteronism are the effect of germline mutations in ion channels, which leads to their inactivation and thus aldosterone overproduction. This article provides a detailed overview and update on the pathophysiology, clinical symptomatology, diagnostics and therapy of different types of this disease.