Pseudohypoaldosteronism type II: the relevance of a challenging diagnosis

We report a case of a young woman with PHA II caused by a novel variant in <i>KLHL3</i>, that highlights the importance of a high level of clinical suspicion for PHA II diagnosis in patients with milder phenotypes and without family history.PHA II should be considered in all patients with low-renin hypertension, hyperkalaemia, hyperchloremic metabolic acidosis and hypercalciuria, regardless of age, clinical features, or family history.Early diagnosis is extremely important because PHA II can be effectively treated with low dose thiazides, avoiding end organ damage onset.