: Standard management protocols for patients with pheochromocytoma (PHEO) have been described in current guidelines. However, the standard management approach for cardiac surgical patients with concurrent clinically silent PHEO has not been established yet. The effects of open heart surgery and cardiopulmonary bypass (CPB) on PHEO presentation are poorly documented. Here, we report in detail the anesthetic management and hemodynamic changes in a case of CPB-assisted pulmonary valve replacement surgery with an incidentally discovered normotensive PHEO. A 55-year-old male with normotensive PHEO underwent an open heart pulmonary valve replacement surgery. Blood pressure (BP) fluctuated with an inherent alternation rhythm during the whole process of the surgery with a peak at 230/130 mmHg and nadir at 40/30 mm Hg. Administration of fluids and vasoconstrictor/vasodilator was used for BP optimization. However, the BP changed intractably with delayed responses to the optimization management. The hemodynamic instability observed in this case indicated that the risk of occurrence and severity of the PHEO crisis did not decrease in cases with previously silent PHEO and may be triggered in any clinical scenario, not in tumor removal procedures alone. Careful preoperative evaluation and preparation with multidisciplinary cooperation are of paramount importance for such patients because open chest cardiac surgery and CPB could complicate the situation. In addition, there is little evidence about the benefits of a combined cardiac surgery with PHEO resection in such a clinical scenario, which validates further investigation.