412Neurenteric cysts are congenital abnormalities usually found within the posterior mediastinum, 1 but can occur in the central nervous system (CNS). They are rare congenital cysts with histological features that indicate an endodermal origin. 2 The cysts are lined by single or multi-layered cuboidal or columnar epithelium. The epithelium may or may not be ciliated and goblet cells are occasionally seen. The epithelium resembles that which lines the respiratory or gastrointestinal tract. 2,3 Central nervous system neurenteric cysts are most commonly found ventral to the spinal cord in the intradural extramedullary space at the level of the cervical or upper thoracic spine. 4 Lumbosacral neurenteric cysts have also been reported. [5][6][7] The majority of intracranial neurenteric cysts occur in the posterior f o s s a 8 , 9 or at the craniocervical junction. 1 0 , 11 S u p r a t e n t o r i a l neurenteric cysts are very rare and none of the cysts in those cases reported to date have been located within the brain parenchyma. 3,[12][13][14][15][16][17][18][19] We report a case of a 35-year-old female who underwent successful excision of an intraaxial right frontal lobe neurenteric cyst. To our knowledge, this is the first case report of A B S T R A C T: B a c k g r o u n d : Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma. M e t h o d s : Apatient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature. Case report: A 3 5 -y e a r-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved. C o n c l u s i o n : Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.RÉSUMÉ: Kyste neurentérique supratentoriel intraparenchymateux. Introduction: Les kystes neurentériques sont des kystes congénitaux du système nerveux central qu'on pense être d'origine endodermique. Nous présentons un cas unique de kyste neurentérique supratentoriel situé entièrement dans le parenchyme cérébral. Méthodes: Une patiente a consulté pour un kyste intraparenchymateux qui a été éventuellement identifié comme étant un kyste neurentérique. Nous faisons une révision de la littérature concernant cette pathologie. Cas clinique: Une femme âgée de 35 ans a consulté pour des céphalées progressives depuis un an et des crises é...