At terminal stage, Sturge–Weber syndrome (SWS) can lead to choroidal calcification due to local chronic ischemia caused by congenital vascular malformation. We report a 51-year-old male with known SWS, who presented to the University of Salzburg eye clinic with chronic pain in the left eye. Upon examination, endophthalmitis, total retinal detachment, and complete loss of vision were diagnosed. Enucleation was performed due to the progression of the disease. During the procedure, ossification of the choroid was seen macroscopically. Histological examination revealed lamellar bone with yellow bone marrow.