A case of pheochromocytoma associated with von Recklinghausen's disease is reported. A 53-year-old man consulted the Department of Dermatology of our hospital because of Dermatoptosis in the breast and abdomen. He asked to resect of the mass. Blood bio chemistry findings were almost within normal limits but only catecholamines in serum and urine were remarkably higher. With a result of other detailed examinations, he was diagnosed as pheochromocytoma. He was referred to our clinic. Laparotomy was carried out under the strict monitoring of the circulation. We diagnosed his clinical status to non-hypertensive type. The tumor was 2.5 x 1.7 x 1.7 cm in size and weighed 10 g. After operation, serum and urine catecholamines returned to all within normal limits. a operation for the dermatoptosis was performed afterwards at the Department of Dermatology.