To our knowledge this is the first reported American case of cutis marmorata telangiectatica congenita (CMTC) combined with other congenital defects, in this instance, Sturge-Weber disease and patent ductus arteriosus. It should be mentioned that CMTC may be associated with a wide variety of malformations. To our knowledge this has not been emphasized in previous American reports. We prefer the original nomenclature of Von Lohuizen and stress the clinical characteristics in diagnosing this disease.