Report of two cases of truncular dermoepidermal skin defect in newborn. The defect concerned the lateral thoracoabdominal area, on both sides of the umbilicus, jointed with a fine linear communication, and have the classical butterfly wind-like shape. Recovery was achieved by centripetal spontaneous cicatrization, only managed with protective vitamin dressing, in about 45 days. There was no clinical evidence of other anomaly. In this occasion, an exhaustive review of congenital skin defects is done. This disease is not frequent, 500 cases are published, principally in the scalp area (vertex). Thoracoabdominal localisation (10%) are symmetrical, spontaneously healing without surgery, in spite of a large defect. Skin defect of extremities (25% of cases) have also a good issue, except in case of associated congenital diseases; on the other hand scalp defects are sometimes complicated with sepsis or bleeding. Discussion emphasize the characteristics of skin defects, the possible pathological association (rare in case of thoracoabdominal defect) and pathogenic hypothesis, not yet definite at all.