Serpiginous choroiditis is a rare, usually bilateral, chronic, recurrent inflammation that affects the outer retina and the inner choroid. The exact etiology of serpiginous choroiditis is still unknown; however, the disease is considered to be autoimmune in nature. The disease usually manifests as geographic, helicoid, or placoid choroiditis lesions that continue to progress in a serpentine manner. Fluorescein angiography, indocyanine green angiography, and optical coherence tomography are very useful in determining the activity of the disease and monitoring the response to therapy. Systemic corticosteroids and immunosuppressive agents are the mainstay of therapy for serpiginous choroiditis. The index chapter illustrates two cases of serpiginous choroiditis with characteristic morphological appearance.