We describe a patient who presented frostbite in extremities in addition to characteristic symptoms, such as severe hypertension, sweating attacks, palpitations and headache. The patient was eventually diagnosed as having single extra-adrenal pheochromocytoma. The frostbite in extremities rapidly resolved after the removal of the tumor as well as other characteristic clinical symptoms. It is speculated that this frostbite might have been induced by severe continuous constriction of peripheral artery and loss of heat by frequent sweating attacks. Regarding cutaneous symptoms in this disease, pallor, acrocyanosis and cold extremities are commonly found. However, it seems that typical frostbite associated with pheochromocytoma has not been reported so far.