Dome-shaped macula (DSM) of high myopia has been described as an inward convexity or bulge of the macular within the concavity of the posterior staphyloma in highly myopic eyes, with the bulge height over than 50 μm, which can be observed by optical coherence tomography. There are three patterns of DSM, including the typical round dome, the horizontally oriented oval-shaped dome and the vertically oriented oval-shaped dome. The pathogenesis of DSM development remains unclear, several hypotheses have been suggested, such as localized choroidal thickening in the macular area, relatively localized thickness variation of the sclera under the macula, resistance to deformation of sclera staphyloma, ocular hypotony and tangential vitreoretinal traction. Vision-threatening macular complications of DSM including serous retinal detachment, choroidal neovascularization, foveoschisis and retinal pigment epithelial atrophy. Clinically, asymptomatic patients with DSM mainly take regular follow-up observation. Appears serous retinal detachment and significant visual impairment, treatment with half-dose photodynamic therapy, supplementary of laser photocoagulation or oral spironolactone may have a beneficial effect. However, more large clinical studies are required to confirm the exact efficacy of these treatments.