Cases of cutaneous amyloidosis which exhibit poikiloderma-like skin changes are extremely rare. There are at least two clinical forms of poikiloderma-like cutaneous amyloidosis (PCA): (1) the ordinary type, and (2) PCA syndrome. The PCA syndrome includes poikilodermatous skin manifestations which may appear early in life and lichenoid papules, both with cutaneous amyloid deposits, frequently associated with light sensitivity and short stature, occasionally with palmoplantar keratosis and blister formation. We carried out an examination of a 5-year-old girl who was compatible with the syndrome.