A 4-year-old boy presented with Goldenhar’s syndrome, Duane’s syndrome and bilateral papilledema. Magnetic resonance imaging of the brain was unremarkable. On lumbar puncture, the cerebrospinal fluid (CSF) pressure measured 36 cm H₂O. CSF examination was normal. The diagnosis of pseudotumor cerebri was made, and treatment with acetazolamide was started. As the papilledema did not resolve, steroids were added to the treatment. Lumbar puncture was repeated after 1 month, and pressure was found to be 30 cm H₂O. Because medical treatment was not effective in lowering the CSF pressure, optic nerve sheath fenestration was performed. Papilledema resolved over the next 2 months. To the best of our knowledge, this is the first case of Goldenhar’s syndrome associated with pseudotumor cerebri.