Background. Adrenal medullary hyperplasia presents with catecholaminergic symptoms, similar to pheochromocytomas. Usually, adrenal medullary hyperplasia is associated with multiple endocrine neoplasia type 2. It has been reported extremely rarely in the literature, and its solitary form is even rarer. Methods. We reported a case of solitary unilateral adrenal medullary hyperplasia with imaging studies including adrenal computed tomography scan and iodine-131-metaiodobenzylguanidine adrenal scan. Pathology was confirmed after unilateral laparoscopic adrenalectomy. Results. A 38-year-old male presented with paroxysmal chest tightness, palpitations, and uncontrolled hypertension. Mildly elevated urine catecholamine levels were found. A slightly enlarged left adrenal gland was observed by adrenal computed tomography, with increased radioactivity on the iodine-131-metaiodobenzylguanidine adrenal scan. Accordingly, the patient underwent laparoscopic left adrenalectomy. His symptoms and blood pressure improved postoperatively, and no adrenal insufficiency was noted during follow-up. Conclusion. Adrenal medullary hyperplasia is rare. It is clinically similar to pheochromocytoma in presentation and diagnostic approach, but its symptoms are milder and urine catecholamine levels are lower. Regarding imaging studies, adrenal medullary hyperplasia commonly appears normal or slightly enlarged on adrenal computed tomography. Thus, during diagnosis, it may be difficult to distinguish between adrenal medullary hyperplasia and a normal adrenal gland with a false-positive iodine- 131-metaiodobenzylguanidine scan, particularly in patients with equivocal symptoms. Herein, the experience related to the diagnosis and management of a patient with adrenal medullary hyperplasia is reported.