Pheochromocytoma is a neuroendocrine tumor that originates from a chromaffin cell located within the adrenal medulla. It is characterized by its ability to secrete catecholamines. Most cases are aged between 30-50 years and is affecting both sexes equally. It is relatively rare with incidence ranging from 2-8 cases per million. Most cases are due to sporadic mutation, but can also occur as part of other autosomal dominant familial disorders chiefly VHL syndrome, MEN2, and NF1. We aimed to review the literature regarding Pheochromocytoma clinical presentation and features in addition to the updated means of management. PubMed database was used for articles selection, and papers were obtained and reviewed. Pheochromocytoma can present in a wide variety of non-specific symptoms which can present a challenge for the early detection of patients. Some of the most common presentations include hypertension, headache, sweating, or palpitations. Treatment is surgical resection with proper pre-operative medical care. A minimally invasive approach using laparoscopic or robotic techniques is advised. Sporadic cases undergo total adrenalectomy while patients with familial disorder have partial adrenalectomy. Other modalities of treatment are reserved for irresectable or metastatic cases.