We describe a patient with malignant pheochromocytoma who developed Jaccoud's-type arthropathy and Raynaud's phenomenon as initial manifestations of malignant pheochromocytoma. Serologic findings included positive antinuclear antibody (ANA) and rheumatoid factor (RF) was also found in this patient. To our knowledge, this is the first time Jaccoud's-type arthropathy with positive ANA and RF has been reported as rheumatic manifestations of pheochromocytoma.