In this study, hypertension and hypertension-mediated organ damage (HMOD) were found to be prevalent in patients with 11β-hydroxylase deficiency (11β-OHD). The observations suggest the importance of early HMOD assessment and optimal glucocorticoid medication in 11β-OHD cases.

• In this retrospective cohort analysis in a single medical center, 28 patients with 11β-OHD were analyzed.

• In this cohort, hypertension and HMOD prevalence was estimated to be 100% and 50%, respectively.

• High blood pressure-induced damage most commonly occurred in kidneys (71.43%), followed by the heart (64.29%), eyes (57.14%) and brain (21.43%).

• The following were identified to be risk factors of HMOD: hypokalemia [odds ratio (OR) 9.16], blood pressure≥180/110 mmHg [OR 22.0] and irregular glucocorticoid use [OR 3.18].

• An independent predictor for HMOD was blood pressure≥180/110mmHg.