Pheochromocytoma, a catecholamine-producing tumor of the chromaffin tissue, may present with various features. Herein, we report case of 66-year-old woman with pheochromocytoma accompanying hyperamylasemia and acute abdomen. She was admitted to another hospital due to myocardial infarction 5 months ago. At that time, pheochromocytoma was suggested on the basis of hormonal studies, but she refused surgical resection. When she came to our hospital, serum amylase level was 703 U/L. Subsequent studies revealed pancreatic type isoenzyme, and elevated lipase level. After normalization of serum amylase level, she undertook laparoscopic adrenalectomy. On pathologic examination, pheochromocytoma was confirmed. There are several cases of pheochromocytoma with hyperamylasemia. In general, the source of hyperamylasemia was thought to be pulmonary endothelial cells under ischemic damage caused by potent vasoconstrictive action of circulating catecholamines. In our case, analysis of isoenzymes and serum lipase level suggest that hyperamylasemia can originate from the pancreas. Thus, pancreatitis also should be considered when serum amylase level is elevated in pheochromocytoma.