Background:Adams-Oliver Syndrome is characterized by the combination of aplasia cutis congenita and limb anomalies. It was initially described in 1945 by Adams and Oliver.Main observations:We report a case of a 10-year-old girl with Adams-Oliver Syndrome with aplasia cutis congenita and limb defects only with no internal organ anomalies.Conclusions:Adams-Oliver Syndrome is a rare multisystem disorder of unknown etiology. It may be presented by isolated aplasia cutis congenita and limb anomalies.