Pheochromocytoma is a rare neuroendocrine tumor that originates from the chromaffin cells of the adrenal medulla. Pheochromocytomas produce hormones called catecholamines in excessive amounts, which are important for regulating body functions and cause secondary hypertension. It can occur sporadically or in inherited syndrome, with a significant number of these tumors carrying germline or somatic mutations in genes. It is recommended for every patient to undergo genetic testing. Most pheochromocytomas are benign, meaning they do not spread to other parts of the body. However, approximately 10% of pheochromocytomas can become malignant and metastasize to other organs, referred to as metastatic pheochromocytoma. Symptoms of pheochromocytoma can be nonspecific and vary from person to person, but hypertension, palpitations, sweating, and headaches, which can occur paroxysmally in attacks, are the most common. Diagnosis of pheochromocytoma involves the patient's medical history, physical examination, laboratory tests, and imaging studies. Confirmation of the diagnosis usually involves urine testing or blood tests to determine the presence of hormones or their metabolites in the body. Treatment of pheochromocytoma typically involves surgical removal of the tumor. Medication therapy, such as alpha-adrenergic blockers, may be recommended before or after surgery to control symptoms and prevent potential complications and tumor recurrence. It is important to monitor patients after treatment for pheochromocytoma to ensure that the tumor does not regrow or metastasize. Regular check-ups, blood tests, and imaging studies may be part of the follow-up monitoring.