Inflammatory diseases affecting the eye, the orbital content, or the endocranium are various. The clinical or radiologic presentation are multiple and their diagnosis can be a real challenge. Most relevant studies or case reports on systemic lupus erythematosus, Wegener's granulomatosis, Churg-Strauss syndrome, giant cell arteritis, Behcet's disease, Takayasu's arteritis, relapsing polychondritis, or inflammatory pseudotumor of the orbit are summarized in the present review.