Background. - Orbital inflammatory lesions may be due to infection, ne
oplasia, systemic disease such as sarcoidosis or be idiopathic. Case r
eport. - A 13 year-old boy had uveitis and symptoms of unilateral peri
globular orbital inflammation, confirmed by computed tomography. Infec
tion and neoplasia were excluded. The serum angiotensin-converting enz
yme level (ACE) was normal. Steroids were effective bur three recurren
ces occurred with bilateralisation of the lesion. Serum ACE level incr
eased making sarcoidosis probable, Moreover renal lesion were later fo
und. Outcome was good with a prolonged steroid administration for ten
months. Conclusions. - Sarcoidosis may be revealed by an orbital infla
mmatory lesion. Steroids are necessary to prevent ocular functional co
mplications.