Ganglioneuroma is a rare, benign tumor originating from the sympathetic ganglion cells. Choroidal ganglioneuroma in association with neurofibromatosis type 1 (NF-1) has been reported in a few cases. This study describes a 21-year-old woman with orbitopalpebral NF, who underwent evisceration for pseudophakic bullous keratopathy, intractable glaucoma, and absolute painful eyes and who was found to have choroidal ganglioneuroma and retrobulbar plexiform neurofibroma on histologic examination. This case, together with the previously described cases, suggests that eyes with choroidal ganglioneuromas may have some common clinical and prognostic characteristics, although this condition cannot be diagnosed preoperatively. In patients with NF-1 who may undergo eye removal surgery, the possibility of choroidal tumors should be kept in mind.