The authors report the case of a six and a half year old girl, with extensive poikilodermatous condition and acrokeratosis, preceded by early blistering and light sensitivity. The sequence of cutaneous symptoms suggests the diagnosis of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler; forty-two cases have already been reported in the literature and our patient would better fit into Kindler's type. The original findings under electron microscopy and direct immunofluorescence (colloid bodies, IgM deposition) that are reported herein are discussed from a pathogenic point of view.