A previous study in an untreated girl with 17 alpha-hydroxylase deficiency syndrome (17-OHDS) indicated that in the absence of cortisol production, ACTH and corticosterone (B) may interact to modulate the brain-pituitary-adrenocortical system. The ACTH-B interplay was further examined in this patient under dexamethasone therapy and after withdrawal of treatment. Plasma ACTH and B were highly correlated (r = 0.89) during two different schedules of dexamethasone treatment and two weeks off treatment at all blood sampling times (morning, afternoon or 24-h period). A logarithmic-sigmoidal relationship seems to exist between ACTH and B secretion. The time course of ACTH-B relationship early after withdrawal of therapy showed good parallelism between these two variables. Plasma B may be a useful guide for monitoring therapy in 17-OHDS. The present study also indicates the ACTH-B interplay feedback set-points.