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Background: Pheochromocytoma is a rare catecholamine-producing tumor of chromaffin cells in the adrenal medulla or of a paraganglion. Typically it presents with sustained or paroxysmal hypertension, severe headaches, palpitations and sweating due to hormone excess. However, the presentation can be variable and can mimic many other diseases. If left undiagnosed or untreated, it can lead to life-threatening consequences. Case Presentation:A 35 year old female with significant past medical history of migraine headaches, poorly controlled hypertension and a recent new onset seizure, presented with progressive worsening shortness of breath and persistent abdominal pain following a gastrointestinal illness. She also reported diaphoresis, cold fingers and toes, abnormal weight gain, and orthostatic symptoms that gradually worsened for two months prior to presentation. Laboratory evaluation revealed lactic acidosis, leukocytosis, and hypokalemia. Subsequently, a CT scan of the abdomen was performed that revealed an adrenal mass with significant elevation in urine metanephrines. As a result, the patient was diagnosed with pheochromocytoma and successfully treated with laparoscopic left adrenalectomy. Conclusion: Pheochromocytoma is a rare but can be life threatening if left undiagnosed. It is of utmost importance for clinicians to keep in mind such unusual presentation of a potentially life threatening tumor. To the best of our knowledge, this is an unusual presentation of Pheochromocytoma with severe lactic acidosis.