Cutaneous epitheliotropic lymphoma describes a form of lymphoma, which may specifically affect the eyelids and adnexal tissues. The somewhat confusing term “mycosis fungoides” has also been used to refer to this condition. Clinical presentation includes thickened, irregular to nodular/proliferative and/or ulcerative dermatitis/blepharitis, and/or depigmentation, which varies in severity and distribution. Mucocutaneous junctions around the eyes and mouth are commonly affected, as are nail beds and/or paw pads, however more generalized dermal involvement may also be present. Treatment comprises systemic chemotherapy as well as the management of secondary bacterial infection where present.