Cutis marmorata telangiectatica congenita (CMCT) is a rare cutaneous vascular anomaly. Two children with monoatrophy of one limb referred for neurological assessment were found to have a segmental form of CMTC. The paediatrician and paediatric neurologist should be alerted to this rare disease in children referred for cutaneous lesion with associated limb asymmetries as distinguished from other neurocutaneous syndromes.