The first, most obvious sign of Sturge-Weber syndrome is a baby born with a facial birthmark. It usually will cover at least one eyelid and the forehead. The mark will vary in color and size. In rare cases, there is no port wine stain. Neurological problems are caused by excessive blood vessel growth on the surface of the brain. Seizures are the most common early problem, often starting by 1 year of age. Developmental delay also may occur in varying degrees.

Other problems may affect:

Eyes: Increased pressure within the eye (glaucoma) can also be present at birth or develop later. This affects about 30% of patients with Sturge-Weber and also can happen without Sturge-Weber syndrome. It most often affects the eye with the port wine stain.

Organs: Other body organs can be affected, although this is rare