The linear naevus sebaceus syndrome (LNSS) is a phakomatosis, characterized in general by a triad consisting of naevus sebaceus of Jadassohn, seizures, and mental retardation. In addition, a broad spectrum of neurological, ophthalmological, skeletal, urogenital and cardiovascular symptoms may be encountered. According to our literature review, seizures and mental retardation were reported in 67 and 61% of cases, respectively. Because ophthalmological abnormalities (59%) and involvement of other organ systems (61%) occur frequently, we advise avoidance of adhering to the classical triad for recognizing or describing LNSS. Gross structural abnormality of the cerebrum or cranium was frequently observed (72%), consisting mainly of enlargement of one lateral ventricle, hemimegalencephaly and hemimegacranium. We report a case of a male patient with the clinical features of LNSS, but without cerebral developmental abnormalities at autopsy examination.