To report the clinical features and management of seven cases of intraocular inflammation caused by Rickettsia infection and review published literature.Rickettsia conorii or Rickettsia spp. infection was diagnosed based on the following criteria: (1) positive serology according to the European Guidelines, (2) titer normalization after specific treatment, and (3) complete resolution of ophthalmic disease and accompanying symptoms after antibiotic therapy.Seven patients were referred for uveitis of unknown etiology. All came from regions where Mediterranean spotted fever is prevalent. One patient met the European guidelines criteria for Rickettsia spp. infection, while the other six cases met the criteria for R. conorii infection. The main symptoms were visual loss, floaters, eye redness, photophobia, and ocular pain. Predominant ophthalmic signs included vasculitis, choroiditis, vitritis, and macular edema. All patients required antibiotic treatment that resulted in the remission of the infection. Doxycycline was the first choice and the only antibiotic used to treat four patients. One patient needed ciprofloxacin as a second antibiotic after not responding to doxycycline. Two patients had doxycycline as a second antibiotic after not responding primarily to sulfonamides (which had been given after 2-3 days of doxycycline gastric intolerance); one of these patients needed ciprofloxacin as a third antibiotic.Intraocular inflammation can occur as the main manifestation of Rickettsia conorii or Rickettsia spp. infection. It should be considered as a differential diagnosis for uveitis especially for patients living in countries where this infection is endemic in the world. Antibiotic treatment remains effective in the management of Rickettsia infection.