Summary:Hodgkin disease with primary manifestation in the orbit is extremely rare, and even when suspected can be very difficult to diagnose. Its clinical and histological presentation can be nearly impossible to differentiate from that of a benign inflammatory process, and it is necessary to utilize immunohistochemical techniques to confirm the diagnosis. This article focuses on a case of nodular sclerosing Hodgkin disease with initial manifestation in the orbit. A comparison of the clinical, histological, and immunohistochemical presentations associated with both Hodgkin disease and benign inflammation is discussed. A brief review of the immunohistochemistry specific for Hodgkin disease is also provided.