Ankylosing spondylitis (AS) is a seronegative spondyloarthropathy, which characteristically results in fusion of the spine and sacroiliac joints. AS can be accompanied by extraarticular manifestations in cardiovascular, pulmonary, neurologic and renal diseases. We herein describe a stable AS patient receiving anti-TNFα. therapy who developed unexplained microscopic hematuria and nephrotic syndrome. Laboratory examinations showed elevated serum immunoglobulin A (lgA) and renal biopsy demonstrated IgA nephropathy. Under immunosuppressive therapy with low-dose steroid and mycophenolate mofetil (MMF) for 6 months, the proteinuria almost remitted completely and renal function remained stable. IgA nephropathy was considered correlated with the pre-existing AS. Anti-TNFα. therapy may also play a role in the exacerbation of IgA nephropathy. This report alerts clinicians to stay watchful for concurrent IgA nephropathy in patients with AS, especially under anti-TNFα. therapy. Corticosteroid combined with MMF is an effective therapeutic regimen for IgA nephropathy in this case report.