Familial Mediterranean Fever (FMF) is an inflammatory autosomal recessive disease characterized by serosal and synovial involvement. Although secondary amyloidosis is the most common of the renal diseases observed with FMF, other glomerular involvements have also been demonstrated. Unfortunately only a few cases about IgA nephritis in patients with FMF have been reported in literature. Here we present a 56-year-old male patient who was diagnosed with chronic IgA nephritis by renal biopsy after detection of intermittent hematuria and proteinuria while he was being monitored for FMF disease. The patient was followed up for 5 years and treated with regular colchicine. Our case shows that amyloidosis is not only the type of renal involvement in patients with FMF. Kidney biopsy should be performed in patients with FMF displaying micro- or macroscopic hematuria with or without proteinuria.