Idiopathic intracranial hypertension (IIH) is a poorly understood condition, and its presentation can coexist with other diseases. Simultaneous IIH and Duane retraction syndrome (DRS) type 1 have never been reported to coexist in an adult patient. Herein, we report a 32-year-old obese female with a history of chronic renal failure who had a renal transplant rejection 6 years prior to presentation and was treated with oral steroids and immunosuppressive medications. She began to experience signs and symptoms of increased intracranial pressure (morning headache and binocular horizontal diplopia) and had limited abduction of one eye on examination. The case was later diagnosed as IIH with DRS type 1.