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We report on 4 cases of brain tumors associated with tuberous sclerosis. In each case the tumor was in the region of Monro’s feramen and was associated with ventricular dilatation. Computer tomography scans, which were performed in 3 cases, revealed well-circumscribed high-density masses which were markedly enhanced by contrast medium. 3 patients underwent craniotomy. Total extirpation was performed in 2 patients. Both of them are doing well postoperatively. However, 1 patient whose tumor had been partially resected died of recurrence 6 years after surgery. The pathology of three tumors was different in each case: plump cell astrocytoma, hemangioma and astroblastoma.

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A 53-year-old male presented with a rare enterogenous cyst in the fourth ventricle associated with repeated ventriculoperitoneal shunt malfunction. Surgical excision of the cyst resolved the shunt problems. Electron microscopy findings of the surgical sample were microvilli covered with electron dense coating materials, basement membrane, and several intercellular junctional devices , suggest ing the cyst was derived from the endodermal structure. The viscous and gelatinous contents of the cyst might be responsible for the shunt malfunction.