Ectrodactyly (lobster claw deformity), ectodermal dysplasia and clefting of the lip or palate, the EEC syndrome, is a rare genetic disorder involving both mesodermal and ectodermal derivatives. Cerebral arterial dolichoectasia, a pathological dilatation and elongation of intracranial vessels, was found in a patient with the EEC syndrome. Her hypertensive cardiovascular disease, mild dementia and trigeminal neuralgia were all related to her dolichoectasia. Although the association of dolichoectasia has not been previously observed with the EEC syndrome, it may be more frequent than currently recognized. Cerebral arterial dolichoectasia and its attendant sequelae could account for some of the unexplained abnormalities described in other case reports of patients with the EEC syndrome.