Abstract: Blepharochalasis syndrome is a rare eyelid disease that often presents in childhood or adolescence. It is characterized by idiopathic localized intermittent painless edema of upper and(or) lower eyelids, which would destroy the tissue structure of eyelid and lead to atrophic, wrinkled periorbital skin and the tortuous and dilated capillaries. Other clinical manifestations include proptosis, prolapse of orbital fat, and lacrimal tissue, etc. The etiology of the blepharochalasis syndrome has not yet to be fully elucidated. It still cannot exclude the participation of genetic factors. IgA and matrix metalloproteinases had been found in the periorbital tissues, which confirm that participation of the immuoreaction which might lead to elastolysis. Elastolysis may ruin the structure and the function of the lymphatic system. All these factors may play a substantial role in the pathogenesis of the disease. The treatment of blepharochalasis divided into the treatment of acute edema and stable period.  (Int Rev Ophthalmol, 2013, 37: 303-307)