Background: Nephrotic syndrome (NS) is characterized by various etiologies that damage the glomerulus. Central serous chorioretinopathy (CSCR) is a retinal disease characterized by neurosensory detachment of the retina. Several case reports have described the relationship between both. Therefore, we try to analyze the epidemiological associations between NS and CSCR using the National Health Insurance Research Database in Taiwan. Methods: Data spanning 14 years were extracted from the National Health Insurance Research Database and sub-grouped. The variables were analyzed using Pearson’s chi-squared test and Fisher’s exact test. The risk factors for disease development with or without comorbidities were examined using an adjusted hazard ratio (aHR). Kaplan-Meier analysis was performed to evaluate the cumulative incidence of CSCR with or without NS. Results: A total of 14 794 patients with NS and 14 794 matched controls without NS were enrolled in this cohort study. The incidence rate of CSCR was higher in the study cohort than in the control cohort (aHR = 3.349, p < 0.001). The overall incidence of CSCR was 44.51 per 100 000 person-years in the study cohort and 33.39 per 100 000 person-years in the control cohort. In both groups, CSCR occurred more frequently in males than in females. Patients aged 40–49, 50–59, and ≥60 years in the study cohort had a significantly higher risk of developing CSCR than those in the control cohort (aHR = 3.445, 5.421, and 4.957, all p < 0.001). NS patient with a 4-week history of steroid usage has a higher risk of developing CSCR (aHR = 2.010, p < 0.001). Conclusion: Our data showed that patients with NS have an increased risk of developing subsequent CSCR. Physician should routinely refer their NS patients to ophthalmologist for ophthalmic evaluation. This is the first nationwide epidemiological study reporting the association between these two diseases. Further studies are needed to clarify this relationship.