Peeling skin syndrome is an extremely rare congenital condition characterized by continuous and spontaneous skin peeling. It has also been referred to as skin shedding, deciduous skin, idiopathic deciduous skin, familial continual skin peeling, and continual skin peeling syndrome. The termpeeling skin syndrome was first coined in 1982 by the researchers Levy and Goldsmith, who reported two patients with a universal ichthyosiform dermatosis since birth. Since then, only 22 cases (14 males, 8 females) have been reported. In the majority of these cases, skin lesions appeared either at birth or early in life. However, similar sporadic cases have been reported in the literature since the 1920s. It is believed to be an inherited autosomal recessive disease, but little else is known about its etiology. Ultrastructural studies and differences among patients suggest that there may be different pathogenic mechanisms behind diagnosis. Specifically, the peeling is due to the separation of the stratum corneum layer just above the stratum granulosum layer of skin.