Type II pseudohypoaldosteronism is an uncommonly reported disorder. The authors recently evaluted a patient who in many thors recently evaluated a patient who in many respects appeared to have this syndrome. He had hyperkalemia, a normal glomerular filtration rate, “normal” serum and urinary aldosterone levels, and low plasma renin activity. In addition, he had a hypertension. Fractional excretion of potassium was reduced in response to sodium chloride loading. However, renal potasium excretion in response to administration of sodium sulfate was normal. Thiazide diuretic restored the serum potassium, the low bicarbonate, and blood pressure to normal. He developed marked natriuresis and kaliuresis in response achieved to exogenous mineralocorticoid has been reported only once previously.