The occurrence of Tolosa-Hunt syndrome (THS) in the setting of discoid lupus erythematosus (DLE) has not been previously reported. We report a case of a 55-year-old Chinese man with established cutaneous lupus who presented with 1 week of worsening blurry vision and ptosis of the left eye with severe headache 2 weeks prior to presentation. His cranial nerve examination was significant for left afferent pupillary defect, red desaturation, ptosis, and oculomotor nerve palsy. He also presented with active DLE lesions. Magnetic resonance imaging brain demonstrated asymmetric thickening and enhancement of the left cavernous sinus consistent with THS. After a 4-week gradual steroid taper his ophthalmoplegia resolved. The unusual occurrence of THS and DLE prompts consideration of nonsystemic autoimmune disorders in diagnosis of THS.