Familial Mediterranean fever (FMF) is an autosomal recessive disease charac-terized by recurrent fever episodes and polyserositis. The most important complication is amyloidosis. Nonamyloidotic nephropathy in FMF is poorly documented. Besides amyloidosis, different types of glomerulonephritis may rarely be seen in FMF patients. A 24-year-old male patient followed up due to FMF was evaluated for macroscopic hematuria and acute kidney injury. The patient was diagnosed as immunoglobulin A nephropathy with renal biopsy. The patient gave a good response to colchicine and steroid treatment. The case reports in the literature about the treatment of the patients with association of FMF and glomerulonephritis are insufficient, and there are no satisfactory epidemiological and treatment outcome reports.