Immunoglobulin A (IgA) nephropathy can be complicated by the nephrotic syndrome in rare cases. Although corticosteroid therapy should be recommended in such cases, the response to steroid treatment has been variable, and spontaneous remission also has been reported without steroid treatment in some cases. We report a retrospective analysis of our experience on the clinical outcomes of nephrotic syndrome in patients with IgA nephropathy, in the nephrology department of a provincial hospital in South Korea. Thirty-three patients with biopsy-proven IgA nephropathy with nephrotic syndrome were enrolled between March 1990 and March 2013. We analyzed data according to demographic, clinical, and laboratory records. The mean follow-up duration was 62 ± 45 months (10-204) in 33 patients. Complete remission occurred in 10 steroid-users and two steroid-nonusers. Partial remission occurred in seven steroid-users, and eight steroid-nonusers. During follow-up, six patients showed progressive deterioration of renal function. Among the IgA nephropathy patients with nephrotic syndrome, 36% and 45% of patients had complete and partial remission, respectively. Steroid treatment may effectively reduce proteinuria. However, spontaneous remission occurs in some cases.