Hypertrichosis cubiti, also named hairy elbows syndrome (HES), is an uncommon variety of congenital, circumscribed hypertrichosis in which a remarkable amount of long vellus hair is localized on the extensor surfaces of the upper extremities. Usually, this condition appears in infancy, increases during early childhood, and regresses spontaneously at puberty. The mode of inheritance is not elucidated, and the condition presents in a sporadic or familial form. In most patients HES is not associated with any other physical abnormalities, although short stature and other developmental disorders have been described in some. An 8-year-old girl affected with HES is reported. The literature on this subject is reviewed, and some clinical and etiopathogenic aspects are discussed.