To report a rare case of multifocal fibrosclerosis involving the retrobulbar tissue and the retroperitoneum. A 59-year-old man presented with bilateral exophthalmos, more serious in the right eye. Right orbital biopsy showed fibrous tissue with inflammatory cells. After the failure of the radiation therapy, a right orbital exenteration was required. Radiological examinations demonstrated a retroperitoneal mass involving the perirenal fat, the aorta, the presacral and the perivesical tissue. Renal function impaired. A left inferior calycoureterostomy and a surgically placement of a right JJ stent were performed. Histological examination of the retroperitoneal biopsies revealed fibrous connective tissue. After 9 years of follow up, there was no change in left visual or renal function (last creatinine: 1.3 mg/dl), and no symptoms or signs of recurrences. This case is the ninth to document the association of orbital pseudotumor and retroperitoneal fibrosis. It is important that both the ophthalmologist and the urologist are aware of the existence of this association, so that suitable treatment can be initiated without delay.