Adams-oliver syndrome with congenital absence of lower lip

Adams-Oliver syndrome is a rare disease characterized by aplasia cutis congenita (ACC) and transverse terminal limb defects (TTLD). Both familial and non-familial cases have been described with predominantly autosomal dominant inheritance and autosomal recessive inheritance in a few. Various systemic involvements lik e central nervous system, cardiovascular system etc. have also been described. We describe here a case of ACC with TTLD characterized by syndactyly of hands and feet without any systemic involvement in a 13 months old female child, without any family history. She also had congenital absence of lower lip, not being described earlier.