Amniotic band syndrome (ABS) was first described by Montgomery in Montgomery (1832). It is a poorly known congenital malformation due to strangulation of the organs by an amniotic fibrous band. Several parts of the body can be affected: for instance, skull, face, neck, trunk and musculoskeletal system. It generally associates three types of anomalies namely, amputations, deformities, and malformations. There are two genuine theories covering this syndrome; the Intrinsic Theory associating the syndrome to a germline defect and the Purely Mechanical Extrinsic Theory related to the amniotic band. These theories have thoroughly tried to explain the disease and the organ involvement (Goldfarb et al., 2009). In the current study, we report a rare case of an open fracture of both leg bones with amniotic disease in a 10-day-old neonate who underwent surgical treatment. In our case, it is a surgical emergency where we try to explain its physiopathology and show how to operate it. We discuss likewise the appropriateness of using the expressions “leg fracture” and “congenital pseudarthrosis of the leg”. Finally, we describe a revised classification by Hall (1982) and Weinzweig (1994) of ABS incorporating a stage with bone involvement.