Cutis marmorata telangiectatica congenita is an uncommon, congenital c
utaneous condition typified by persistent cutis marmorata and other as
sociated abnormalities. Progressive neurologic complications are gener
ally not a feature of the disorder. A case is reported of cutis marmor
ata telangiectatica congenita associated with diffuse cerebrovascular
infarcts at 7 months of age. Moyamoya-like vascular abnormalities were
demonstrated in addition to the factor V Leiden mutation, a congenita
l hypercoagulable disorder. This novel case illustrates the importance
of evaluating children with strokes for congenital thrombophilic diso
rders. (C) 1998 by Elsevier Science Inc. All rights reserved.