Background Most cases of orbital sarcoidosis are associated with a sys
temic sarcoidosis. Patients and methods A 67-year-old woman suffered f
rom an orbital mass on the right side, which led to disturbance of the
ocular motility. Slight improvement was achieved by the administratio
n of systemic steroids. Results Histologically a chronic granulomatous
inflammation was revealed in the biopsy of the orbital mass. The susp
ected diagnosis was sarcoidosis, but three conventional chest X-rays w
ithin 10 months and the serum angiotensin-converting-enzyme were norma
l. Suprisingly a computertomography of the chest showed mediastinal ly
mphomas. Conclusion Granulomatous orbital inflammation without any loc
al cause or other systemic granulomatous disease strongly suggests a s
ystemic sarcoidosis. In case of missing lymph node enlargement in conv
entional chest X-ray computertomography should be performed.