Double inversion recovery (DIR) imaging provides two inversion pulses that attenuate signals from cerebrospinal fluid and normal white matter. This review was undertaken to describe the principle of the DIR sequence, the clinical applications of 3D DIR in various central nervous system diseases and the clinical benefits of the 3D DIR compared with those of other MR sequences. 3D DIR imaging provides better lesion conspicuity and topography than other MR techniques. It is particularly useful for diagnosing the following disease entities: cortical and subcortical abnormalities such as multiple sclerosis, cortical microinfarcts and cortical development anomalies; sulcal abnormalities such as meningitis and subacute/chronic subarachnoid hemorrhage; and optic neuritis caused by multiple sclerosis or neuromyelitis optica.