We report a case of a ventricular dermoid cyst associated with a dermal sinus connected with the ethmoidal cells in a patient who developed rapid symptoms of raised intracranial pressure. Computed tomography showed a cystic mass in the right lateral ventricle with a hydrolipidic image in the left frontal horn of the ventricle and associated hydrocephalus. Magnetic resonance imaging showed a heterogeneous T1 hyperintense mass with a fistulous tract communicating with the ethmoid cells. A cerebrospinal fluid ventriculo-peritoneal shunt was initially established, which required further revision. A right sided transventricular approach was undertaken in a second stage, allowing resection of a dermoid cyst. Obliteration of the dermal sinus tract was obtained using pericranial duraplasty. Clinical and imaging features are discussed. The need for total resection including the tumor capsule and occlusion of the fistula are emphasized if recurrence and infection are to be prevented.
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