Abstract: Keratosis lichenoides chronica (KLC) is a rare keratotic disease, which should be differentiated from linchen planus, lichen planus-like keratosis, lupus erythematosus, et al. The treatments include topical calcipotriol, PUVA, NB-UVB, photodynamic or oral tretinoin, which can alleviate the disease. In this article, the update of etiology, pathogenesis, clinical manifestation, diagnosis and treatment of KLC is reviewed.