Aim: To describe a rare case of bilateral pheochromocytoma in a young male presenting with two episodes of myocardial infarction. Background: Pheochromocytoma is a catecholamine-producing tumor which arises from the adrenal medulla or rarely from an extra-adrenal site. It may present with a wide range of clinical symptoms and signs, ranging from headache, palpitations, and paroxysmal hypertension to rare events such as cardiomyopathy, cardiogenic shock, seizures, and intracranial bleeding. Case description: We report a case of a 39-year-old male who presented to us with complaint of pain abdomen, palpitation, episodic headache, and shortness of breath for last 1 year. He also had history of two episodes of myocardial infarction during the last 9 months. After careful history, examination and investigations he was diagnosed as a case of bilateral functional adrenal pheochromocytoma with secondary cardiomyopathy. Bilateral open transperitoneal adrenalectomy was done and patient improved symptomatically. Histopathology of the specimen confirmed the diagnosis of bilateral benign pheochromocytma. Owing to this rare presentation of a very rare tumor this case is being reported here. Conclusion: Functional pheochromocytoma rarely presents with cardiomyopathy and myocardial infarction. There should be a high level of suspicion if a young patient presents with myocardial infarction in the normal coronary angiography. Clinical significance: This case illustrates an uncommon presentation of pheochromocytoma presenting with two episodes of myocardial infarction in a young man with normal coronary angiography. A high suspicion index for pheochromocytoma is required in such cases.