Serpiginous choroiditis (SC) is infrequent, chronic and posterior uveitis displaying a geographic pattern of choroiditis easy to recur. Studies reveal that the active lesions of inflammatory processes are mainly localized to the choriocapillaris and retinal pigment epithelium cells. SC may manifest with variable features, although a creeping pattern of choroiditis, extending from the juxtapapillary area, with grayish yellow discoloration. Fundus fluorescein angiography, indocyanine green angiography, fundus auto-fluorescence and optical coherence tomography are helpful to diagnose atypical SC. In addition, these image examinations can evaluate the activity and progression of lesion, and detect any complication that might occur. SC is mainly distinguished from multifocal SC related with tuberculosis or virus and etc. Pathogenesis is unclear, an organ-specific autoimmune inflammation or infection seems likely to be the underlying process. It is mainly using glucocorticoid with immunosuppressant therapy at present. Timely and effectively control inflammation can effectively prevent vision loss, choroidal neovascularization and choroidal scar in SC patients.