Abstract: A 50-year-old female presented with yellowish brown proliferative tumor around the orbit of both eyes for more than 10 years. Dermatological examination showed that there were yellowish brown proliferative masses around the left orbit and inside and outside the right orbit, with mild telangiectasia on the surface, solid touch and clear boundary. Ophthalmic examination revealed retinal pigment epithelium detachment in the left eye. Laboratory examination found anemia and thrombocytopenia, and there was no abnormality in serum immunoelectrophoresis. Histopathology showed diffuse infiltration of lymphocytes, epithelioid cells and foam like cells in the dermis and subcutaneous tissues, and focal necrosis was seen. The diagnosis of necrobiotic xanthogranuloma was made.

Key words: necrobiotic xanthogranuloma, retinal pigment epithelium detachment, anemia, thrombocytopenia