We describe a 56-year-old white man who presented with gradual and progressive visual loss and subsequent hypertension and pedal edema. A computed tomographic scan of the orbits showed bilateral diffuse retrobulbar masses, and an abdominal computed tomographic scan showed a diffuse retroperitoneal mass invading the aorta, ureters, and inferior vena cava. Biopsies of the orbit and abdomen confirmed a heterogeneous cell population and marked fibrosis consistent with a sclerosing inflammatory process. Bilateral sclerosing orbital inflammatory disease should cue the physician to suspect coexisting systemic disease. This report is the fourth to document bilateral sclerosing orbital inflammatory disease and the second to have obtained biopsies of the orbit and abdomen showing histologic similarities.