Objective: Primary bilateral macronodular adrenocortical hyperplasia (PBMAH), characterized by benign adrenocortical macronodules and variable levels of cortisol excess, can also lead to an excess of mineralocorticoids, although there is only anecdotal evidence. To assess the prevalence and characteristics of primary aldosteronism (PA) in patients with PBMAH. Design and method: We conducted a systematic review according to the PRISMA guidelines. We reviewed single case reports and case series of patients with PBMAH and PA in Pubmed, Embase and Cochrane from database inception to December 2023. PBMAH was defined radiologically as at least 1 bilateral adrenal nodule of at least 10 mm. Findings were compared to patients with PBMAH without PA derived from LMU Hospital and literature. Results: 1018 articles were screened, and 36 articles were assessed for eligibility. We identified 18 articles (8 single case reports and 10 case series), resulting in 68 cases with PBMAH with PA. Of those, 12 had cosecretion of cortisol. The prevalence of PA in PBMAH, in six studies (166 patients), ranged from 2-44%. The median age at diagnosis was 51.5 (47.5-56.2). The majority of patients with PBMAH and PA were male compared to patients with PBMAH without PA (59% vs 39%, p= 0.0295). At baseline, the mean arterial pressure was 112 mmHg (113-133) and the median duration of hypertension was 108 months (48-135). The serum aldosterone concentration and plasma renin activity were 253 ng/l (159-436) and 0.23 ng/ml/h (0.16-0.44), respectively. Adrenal nodules were smaller in patients with PBMAH with PA compared to patients with PBMAH without PA (22 vs 30 mm, p=0.0498). Treatment modality was reported in 30 cases: 28 patients underwent adrenalectomy (two bilateral, two subtotal, and 24 unilateral) and two received mineralocorticoid receptor antagonists (MRA). In comparison, fewer patients with PBMAH without PA underwent adrenalectomy (54/106, p=0.0005). Conclusions: PA in PBMAH appears to be more frequent than previously thought. Multicenter studies will help to clarify the association between these conditions and the most appropriate treatment modality.