Pseudotumor was diagnosed in six patients aged three to 38 years during an eight year period. The diagnosis was based on headache, choked disks and normal cerebrospinal fluid and CT scans. Other symptoms were visual impairment, VIth nerve palsy and ataxia. Sagital sinus trombosis was excluded by angiography or magnetic resonance imaging. Pharmacological treatment with digitoxin, acetazolamide, furosemide or corticisteroids reversed clinical symptoms and signs in two patients. In the other four, shunt surgery with cerebrospinal fluid diversion to the right atrium or the peritoneal cavity rapidly reversed clinical symptoms and signs.