Orbital inflammation initially presented in a patient who five years later developed renal obstruction from retroperitoneal fibrosclerosis. Biopsy revealed infiltration of chronic lymphocytic cells in the orbit and dense fibrous connective tissue in the pelvis. Episodes of proptosis were controlled with systemic steroids and non-steroidal anti-inflammatory agents. The hydronephrosis was relieved by intraperitonisation of the ureters. Similarities to previously reported cases are discussed. This case shows the importance of searching for systemic disease in patients with orbital inflammatory disease and informing the patients of the possibility of future systemic manifestations of their disease.