Sympathetic ophthalmia is a rare, bilateral granulomatous uveitis that occurs after either surgical or accidental trauma to one eye. The etiology is still not completely clear, but evidence suggests that sympathetic ophthalmia represents an autoimmune inflammatory response against choroidal melanocytes mediated by T cells. Key features are vision impairment and symptoms associated with inflammation. The diagnosis is based on a history of previous ocular trauma or surgery and clinical findings. Differential diagnoses include other causes of granulomatous uveitis, such as Vogt-Koyanagi-Harada disease, sarcoidosis, tuberculosis, and syphilis. Depending on the suspected etiology, treatment of sympathetic ophthalmia consists of systemic anti-inflammatory agents, including corticosteroids and immunomodulating drugs. The role of enucleation after the diagnosis of sympathetic ophthalmia remains controversial. Visual prognosis is reasonably good with prompt appropriate wound repair and immunomodulatory therapy. As the occurrence of sympathetic ophthalmia is probably more frequent following vitreoretinal surgery, more attention has to be paid to this potentially bilateral blinding disorder.