A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome.

Background: Epidermal nevus syndrome is a multi-system disease with a wide spectrum of clinical presentation. Numerous specialists may be required to address its extra cutaneous manifestations.

Main Observations: We report a severe case of epidermal nevus syndrome involving the oral cavity, pharynx, and central nervous system in addition to disfiguring skin lesions.

Conclusions: Dermatologists are in a unique position to first render the diagnosis of epidermal nevus syndrome for young patients and ensure appropriate follow-up.