Abstract This report describes a child who presented at birth with a large congenital scalp nevus that was successfully serially excised in three stages during the second year of life. As the child's development progressed, delayed milestones became apparent and seizures developed. The child was diagnosed with linear nevus sebaceous syndrome (LNSS). LNSS is a subtype of epidermal nevi associated with seizures, psychomotor retardation and neurocognitive delays. It is essential for pediatricians to be aware of the existence of a spectrum of epidermal nevus syndromes and make timely treatment referrals.