Abstract:A 9‐year‐old girl was diagnosed as having a linear sebaceous nevus syndrome (LSNS). The nevus sebaceus was located on the face, and the girl also had nevoid hypertrichosis on the neck, sensorineural deafness, partial anodontia, blocked tear ducts, labiopalatoschisis, and an area of micropolygyria in the left encephalic (cerebral) hemisphere. Electroencephalographic alterations were detected, but they were not accompanied by a history of seizures; furthermore, the child was not mentally retarded. This phenotypic pattern of LSNS is unusual for the rarity of associated abnormalit