Aldosterone is the principal circulating mineralocorticoid in humans, and aldosterone synthesis normally occurs in the face of volume depletion and renin stimulation. In primary and secondary aldosteronism, aldosterone synthesis continues despite volume expansion and causes hypertension. Other steroid hormones that are aldosterone and cortisol precursors also activate the mineralocorticoid receptor and cause hypertension when overproduced. Mineralocorticoid synthesis in these pathologic states can be constitutive or driven by pituitary adrenocorticotropic hormone (ACTH), due to genetic defects that cause disordered steroid synthesis or catabolism. This review focuses on uncommon forms of ACTH-dependent mineralocorticoid excess states associated with hypertension.