Adams–Oliver Syndrome with Widespread CMTC and Fatal Pulmonary Vascular Disease

: We report a neonate with cutis marmorata telangiectatica congenita and clinical features of Adams–Oliver syndrome in association with severe pulmonary vascular disease. We provide an overview of cutis marmorata telangiectatica congenita, distinguishing it from cutis marmorata, a common and benign physiologic cutaneous disorder seen in neonates. We highlight the need for thorough medical evaluation in cutis marmorata telangiectatica congenita to exclude associated congenital anomalies.