A unique case of a 15-year-old girl with nevoid basal-cell carcinoma (NBCCS), who presented with uncontrolled temporal lobe epilepsy due to neuronal heterotopia, is reported from the Royal Melbourne Hospital, Australia. Seizures began at age 13 months, often precipitated by fever. She had a dermoid cyst removed from her nose, multiple dental cysts, and seborrheic keratosis of the scalp. Growth increased dramatically at age 9 years, and seizures became refractory to treatment. Chest X-ray showed cervical spina bifida occulta and fused ribs. Head CT showed calcification of falx cerebri and tentorium. MRI showed a lesion in the left anterior temporal lobe. EEG recorded seizure activity in the left frontotemporal areas. Temporal lobectomy specimen showed neuronal heterotopias and cortical dysplasia. After surgery, she was seizure free at 6 months follow-up. 
COMMENT. Nevoid basal-cell carcinoma syndrome presents at puberty with multiple basal-cell carcinomata, palmar and plantar pits, odontogenic painful cysts of the mandible, facial paresthesia, macrocephaly, large stature, frontal bossing, broad nasal bridge, narrow shoulders, fused ribs, Polydactyly, bone cysts, calcified falx, cervical spina bifida occulta, odontoid agenesis, corpus callosum agenesis, meningioma, and medulloblastoma. Medulloblastoma, occurring in 3-5% of cases, may precede the development of other manifestations of the syndrome, and may have a better prognosis than general. Patients with seizures should be investigated for neuronal heterotopias, which may be treated surgically. The syndrome is autosomal dominant and the gene is localized to chromosome 9q22.