We report a young female with autosomal dominant polycystic kidney disease associated with right adrenal adenoma. Refractory hypertension and hypokalemia were the findings that led to this diagnosis. The diagnostic approach included plasma potassium and aldosterone levels, plasma renin activity, captopril test, saline infusion test, NP-59 scintigraphy, computed tomography, and magnetic resonance imaging. Adrenalectomy completely normalized hypertension and hypokalemia in this patient.