Objective: Primary aldosteronism is one of the most common causes of secondary hepertension. We present here a case of primary aldosteronism in a 38-year-old Chinese male with a 6-year history of uncontrolled hypertension that evaded diagnosis until an attack of rhabdomyolysis due to profound hypokalemia. We also present the results of a comprehensive review of the current literature.Methods: We describe the presentation and symptoms of the patient and review the relevant literature. A thorough literature search disclosed 15 further cases of rhabdomyolysis due to undiagnosed primary aldosteronism reported between 1978 and 2013. We summarized the clinical features, treatments, and outcomes of those cases.Results: Many of the cases presented with previous hint of primary aldosteronism, yet they evaded diagnosis. Three of these patients developed acute renal failure. All patients survived, and many of them were restored to normal blood pressure and normal serum potassium levels after adrenal surgical interventions. Conclusion:Primary aldosteronism can lead to severe acute consequences of rhabdomyolysis via hypokalemia. It is important to alert physicians to the need for hyperaldosteronism workup when a rhabdomyolysis patient is found to be hypokalemic. (AACE Clinical Case Rep. 2015;1:e21-e27) Abbreviations: ARR = aldosterone to renin ratio; AVS = adrenal venous sampling; CPK = creatinine phosphokinase; CT = computed tomography; PA = primary aldosteronism; PAC = plasma aldosterone concentration; PRA = plasma renin activity