We describe a patient with positive antinuclear and anti-Smith antibodies, proteinuria, and thrombocytopenia suggesting systemic lupus erythematosus (SLE). During hospitalization, the patient developed labile hypertension, tachycardia, and intermittent fever. A computer tomography scan of the abdomen showed an extraadrenal mass, which was confirmed as a pheochromocytoma. After removal of the pheochromocytoma, the patient's symptoms resolved and her serology normalized. Previous case reports describe SLE patients with adrenal pheochromocytomas that presented many years after the diagnosis of lupus. This is a novel case of pheochromocytoma discovered at the onset of SLE, with resolution of SLE manifestations shortly after its removal.