IntroductionAdams-Oliver syndrome (AOS) is a rare congenital disorder characterized by congenital cutaneous aplasia of the scalp and terminal transverse limb anomalies (1). The lesions of congenital cutaneous aplasia are generally located on the midline of the parietal or occipital regions, where they may be associated with a parietal bone defect, but may also appear on the abdomen or limbs(1–3). First described by Adams and Oliver in 1945, this syndrome also presents severe forms of expression, including central nervous system anomalies, cardiovascular disease, and gastrointestinal malformations (1,4–6). Several modes of transmission have been described: autosomal dominant, autosomal recessive, and sporadic mutations (7–9). Six genes responsible for Adams-Oliver syndrome have been identified, including ARHGAP31, RBPJ, NOTCH1, DLL4, DOCK6, and EOGT (5,10,11). We report the case of a term newborn with severe intrauterine growth retardation (IUGR) presenting with typical signs of Adams-Oliver syndrome without associated visceral complications. This syndrome remains rare and poorly described in the scientific literature in Burundi.