Case studies of patients with familial schizophrenia may help to define the pathophysiology of this illness and indicate potential candidate genes for genetic linkage studies. In this regard, the clinical, radiological and pathological assessments of a 39-year-old affected man from a pedigree with familial schizophrenia are presented. Brain imaging with CT indicated moderate cortical atrophy, particularly of the temporal lobes. Neuropathological examination revealed granular ependymitis, indicating possible past ventricular pathology. Granular ependymitis was reported to occur in genetic developmental disorders with neuronal migration abnormalities. In the present case, heterotopic clusters of neurons were visualized in the entorhinal cortex, suggesting that temporal lobe development was not entirely normal. This case study suggests that genetic factors could be investigated further as one possible aetiology of certain neurodevelopmental abnormalities observed in schizophrenia.