Background: Ocular involvement, although common in sarcoidosis, granulomatous infiltration of the orbital tissue remains very rare. Case Presentation: We describe the case of a 55-year-old man who presented bilateral protrusion of eyeballs that had been evolving for 1 year. Clinical examination found an axial and non-pulsatile bilateral exophthalmos with cervical lymphadenopathy. CT and MRI showed infiltration of oculomotor muscles. Biopsy of salivary glands showed chronic inflammatory granulomatous infiltration without caseous necrosis. There was a significant rapid improvement with the use of a steroid as primary therapy. Conclusion: Sarcoidosis may be a part of exophthalmos causes, after ruling out the main causes which include: Graves' disease, carotid-cavernous fistula, tumors, and infectious causes.

Keywords: Exophthalmos, systemic sarcoidosis, orbital involvement, corticosteroids.