This 9-year-old boy had two circular areas of aplasia congenita of the scalp in the territory of the first division of the trigeminal nerve and an ipsilateral parieto-occipital leptomeningeal capillary venous angioma. He had an associated giant aneurysm of the distal posterior cerebral artery on the same side as the vascular malformation. The patient had a long history of seizures and presented with intracerebral bleeding. This case may be considered as a variant of Sturge-Weber syndrome. Further observations must be added to consider this entity as a separate neurocutaneous disease.