To evaluate the distribution of skin lesions, clinical features, and associated abnormalities in children with cutis marmorata telangiectatica congenita at onset and during follow-up.Retrospective survey of the available medical data with an average follow-up of 1 year 5 months (range, 0-7 years).Pediatric Dermatology Unit (Department of Dermatovenereology) of the Sophia Children's Hospital in Rotterdam, the Netherlands.The diagnosis of cutis marmorata telangiectatica congenita was clinically established in 35 patients between July 1988 and February 1997. In 33 cases, the typical mottled, blue-violet pattern was present from birth and was readily visible at rest. In 2 cases, the skin lesions initially appeared less reticulated, mimicking a capillary malformation.The skin lesions were almost generalized in 4 children (11%), whereas they were more localized in the other 31 children (89%). Associated anomalies, usually minor and sometimes questionable, were noted in 80% of the patients. Most patients showed a definite improvement of their mottled vascular skin lesions within 2 years. The lesions had totally disappeared, or only faded residual lesions remained.We believe that cutis marmorata telangiectatica congenita is a relatively mild condition. The prognosis is usually good, with minor associated anomalies. Improvement of the mottled, vascular pattern is usually observed within 2 years. We recommend careful clinical examination of all patients to exclude any associated anomalies. Patients should be referred to a neurologist or an ophthalmologist only if symptoms are present or if vascular lesions are present around the eyes.