Restrictive dermopathy is a rare autosomal recessive disorder characterized by extreme tautness of the skin causing restricted intrauterine movement and a fetal akinesia deformation sequence. It is uniformly mostly neonatally fatal. The diagnostic findings are skin tautness with near absence of the dermal elastic fibers, usually with no or only minor anomalies of the internal organs. The exact pathogenetic mechanisms are still not known. Fewer than 50 cases have been reported. We report on a case of restrictive dermopathy and discuss the differential diagnoses.