Blepharochalasis is a rare eyelid disorder that often presents in childhood or early adolescence. It is characterized by exacerbations and remissions of painless edema of the upper and occasionally lower eyelids. Although the average duration of attack is only two days, multiple attacks eventually lead to atrophic, wrinkled, and discolored periorbital skin. Other clinical manifestations include ptosis, acquired forms of blepharophimosis, lower lid retraction, pseudoepicanthal folds, proptosis, prolapse of orbital fat, and lacrimal tissue. The etiology of blepharochalasis has yet to be fully elucidated, but histpathologic examinations indicate that elastolytic activity, immunoglobulin A (IgA), and other inflammatory processes might play a substantial role in the pathogenesis of the disease. The treatment of blepharchalasis is primarily surgical, and therefore understanding the natural history is essential to avoid overcorrection and recurrences after surgery. In this review we present the clinical characteristics, differential diagnosis, and treatment options of blepharochalsis.