Objective To observe the clinical characteristics and fundus imaging features of unilateral acute idiopathic maculopathy (UAIM). Methods Retrospective cases series. Six eyes of 6 patients with UAIM were included in this study. There were 4 males and 2 females. All patients underwent best corrected visual acuity (BCVA), fundus color photography, spectral domain optical coherence tomography (SD-OCT) and fundus fluorescein angiography (FFA) examinations. 3 patients underwent indocyanine green angiography (ICGA) examination. No intervention was given in all patients after diagnosis. The mean follow-up was 6.3 months. BCVA, fundus color photography, SD-OCT and FFA were performed in follow-up. Results The ocular symptoms included unilateral sudden decreased vision, metamorphopsia and central scotoma. All patients had a yellow-white lesion involving the macular and irregular small bleeding was seen in 3 eyes. Irregular hyper-fluorescence in macular area was seen in all patients, some with the hemorrhages showed blocked fluorescence at the early stage of FFA and fluorescence storage at the late stage. ICGA showed that hypo-fluorescence in macular area continually. SD-OCT showed that subretinal fluid in the macular and lost the ellipsoid zone. During the follow-up period, the subretinal fluid was absorbed spontaneously in one month. The ellipsoid zone structure recovered gradually and the visual acuity were normal. At the time of the latest follow-up, the BCVA was 0.8 in 4 eyes, 1.0 in 2 eyes. Conclusions UAIM is featured with a unilateral, acute, transient and exudative lesion in the macular, which can be spontaneously resolved; It mainly affects outer retina.