[Inflammatory pseudotumor of the orbit and suspected sarcoidosis].

Background: Orbital inflammatory lesions may be due to infection, neoplasia, systemic disease such as sarcoidosis or be idiopathic.
Case Report: A 13 year-old boy had uveitis and symptoms of unilateral periglobular orbital inflammation, confirmed by computed tomography. Infection and neoplasia were excluded. The serum angiotensin-converting enzyme level (ACE) was normal. Steroids were effective but three recurrences occurred with bilateralisation of the lesion. Serum ACE level increased making sarcoidosis probable. Moreover renal lesion were later found. Outcome was good with a prolonged steroid administration for ten months.
Conclusions: Sarcoidosis may be revealed by an orbital inflammatory lesion. Steroids are necessary to prevent ocular functional complications.