This systematic review of biopsy proven cases of non specific orbital inflammation and sclerosing variant from 2010-2016 attempts to elucidate new findings regarding the demographic profile of the diseases. Discussion of histopathology is emphasized, as to describe the most common presenting features of non-specific orbital inflammation as well as to discuss its molecular profile. This review also discusses the therapeutic approach and discusses the “steroids vs. biopsy first” controversy regarding non-specific orbital inflammation. The incidence of IgG4 and its relationship to non-specific orbital inflammation is also discussed.