We report a case of an 11-year-old girl with bilateral panuveitis in association with pseudotumor cerebri. The patient underwent complete ophthalmologic, neurologic, and laboratory evaluations and was treated with therapy for pseudotumor cerebri. The patient met the diagnostic criteria for pseudotumor cerebri and also had panuveitis. Symptoms and findings of pseudotumor cerebri and panuveitis improved significantly after combination therapy of oral acetazolamide and weight reduction. The index case illustrates that pseudotumor cerebri can be associated with panuveitis. Therapy for pseudotumor cerebri might also help with the resolution of uveitis.