The diagnosis of epidermal nevus syndrome
should be considered when there is a nevus with associated developmental abnormality of the central nervous system, eyes, or musculoskeletal systems.
Syndromic hemimegalencephaly (for example, epidermal nevus syndrome
and Proteus Syndrome) is associated with other features which include hemicorporal hypertrophy of the ipsilateral part of the body.
Although this anomaly usually affects dental tissues, case reports have documented the presence of regional odontodysplasia with epidermal nevus syndrome
[2, 3], hypoplasia of the affected side of the face , hypophosphatasia , hydrocephalus and mental retardation , and ipsilateral vascular nevi [4, 7].
The clinical Differential diagnosis like Proteus syndrome, Klippel-Trenaunay syndrome, Epidermal nevus syndrome
and other vascular malformations were considered and the relevant investigations were carried out.
look into epidermal nevus syndrome
(ENS) (a rare disorder characterized by distinctive, colored birthmarks on the skin; neurological and skeletal abnormalities may also occur).
Epidermal nevus syndrome
: a review and case report.
Epidermal nevi and the epidermal nevus syndromes
: a review of 233 cases.
Occasionally these may be familial and may occur in association with Goldenhar syndrome or epidermal nevus syndromes
.  Four categories of ocular choristomas described are dermoid, dermolipoma, complex choristoma and single tissue choristoma.