Objective To summarize the neurologic manifestations, diagnosis, treatment and prognosis of atypical primary aldosteronism (PA) with paroxysmal dizziness which is not associated with elevated blood pressure. Methods and Results The clinical data of five patients diagnosed as atypical PA with paroxysmal dizziness but without elevation of blood pressure were retrospectively analyzed. Among the five patients, one was male and four were females, aged 50-95 years, with onset of paroxysmal dizziness. The disease duration was 20 d to 1 year, with the frequency ranged from 0.5 times per month to 1-2 times a day, lasting for a few seconds to 30 min every time, with concomitant symptoms including headache, vertigo, nausea, tinnitus, fatigue, hyperidrosis, palpitation, etc. The complexion may turn red or the head and face may feel febrile consciously, especially during the posture changes or the activity, and there was no obvious discomfort during interictal period. Two patients had a history of hypertension, and three patients had blood pressure fluctuations after 24 h ambulatory blood pressure monitoring. The serum potassium of the five patients was in the normal range on admission, and it was decreased in two patients with dizziness onset. Adrenal contrast-enhanced computed tomography showed left adrenal hyperplasia in one case and no significant abnormalities in the other four cases. The aldosterone renin ratio (ARR) increased, and PA was diagnosed by combining the result of adrenal computed tomography. All the five patients were treated with spironolactone, and their dizziness was significantly improved after regular clinical follow-up. Conclusion Atypical PA with paroxysmal dizziness but without elevated blood pressure is rare, and further study is needed to better understand the disease. To avoid misdiagnosis and missed diagnosis, the history of hypertension should be inquired, and the aldosterone should be valued. Hypokalemia could not be used as a good indicator for PA screening due to low sensitivity and specificity.