Secondary (symptomatic) narcolepsy is rare. We report a subependymoma of the fourth ventricle associated with narcolepsy. The patient was a 50-year old woman with a long history of narcolepsy who died of colonic carcinoma with no cerebral metastasis. She was positive for HLA-DR2. At autopsy there was a tumour dorsal to the fourth ventricle which involved the midbrain tectum and rostral pons. Histologic examination of the tumour confirmed it to be a subependymoma.