Morineau et al. described a French boy with compound heterozygous mutations (c.431 A > T and c.1099_1101del) in HSD11B2 causing severe hypertension, low to normal serum potassium, low plasma renin, and aldosterone levels, as well as markedly reduced 11βHSD2 activity [10]. The gene discussed is HSD11B2; the disease is hypertensive disorder.