A 48-year-old woman suffering from chest and lumbar pain was referred to our clinic for treatment. She had a history of von Recklinghausen's disease with multiple café-au-lait spots and neurofibromatosis. Computed tomography demonstrated a large tumor in the left adrenal gland and a small lesion in the liver, with the levels of plasma and urinary catecholamines being elevated. 131I-metaiodobenzylguanidine (MIBG) scintigraphy showed abnormal accumulations in the left adrenal tumor and multiple-bone lesions. A diagnosis of malignant pheochromocytoma with liver and bone metastases was made, and the patient received chemotherapy. Seven months after the diagnosis of malignant pheochromocytoma, she died of pulmonary edema due to disease progression. Autopsy revealed malignant pheochromocytoma with liver, lung, bone and lymph nodes metastases. We reviewed the literature on pheochromocytoma associated with von Recklinghausen's disease. To our knowledge, only 7 cases of malignant pheochromocytoma associated with von Recklinghausen's disease have been reported in Japan.