Purpose: Macular edema, serous retinal detachment, and retinal pigment epithelial detachment have been reported in patients with nephrotic syndrome. However, there is limited data about macular thickness in children with nephrotic syndrome. The aim of this study was to compare the mean macular thickness in children with nephrotic syndrome and in a control group and to correlate it with visual acuity and level of proteinuria. Methods: The comparative cross-sectional study included 66 children aged 6 to 17 years with nephrotic syndrome and healthy control seen in two tertiary centers in Malaysia. We recorded demographic data, as well as visual acuity, level of proteinuria, and the mean macular thicknesses in both groups. The mean macular thickness was measured using Stratus optical coherence tomography according to nine areas of the Early Treatment Diabetic Retinopathy Study map. Results: The mean foveal thickness was 238.15 ± 22.98 µm for children with nephrotic syndrome and 237.01 ± 22.60 µm for the control group. There was no significant difference in the mean macular thickness between the groups (p = 0.843). A significant correlation with visual acuity was observed in the superior outer macula (r = –0.41, p = 0.019), the nasal outer macula (r = –0.41, p = 0.019), and the inferior outer macula (r = –0.40, p = 0.021). There was no significant correlation between the mean macular thickness and level of proteinuria (p = 0.338), although those with higher levels of proteinuria demonstrated a trend towards increased macular thickness. Conclusions The mean macular thickness in children with nephrotic syndrome was similar to that of healthy children. A significant correlation between the mean thickness of the outer macular layer and the presenting visual acuity was observed. There was no correlation between the mean macular thickness and the level of proteinuria.