The aim of this study was to investigate the effect of colchicine therapy on the outcome of amyloid nephropathy of familial Mediterranean fever (FMF) in childhood. The diagnosis of amyloidosis type AA was confirmed by renal biopsy in 38 patients. During a mean follow-up period of 30.5 months (range 6-88 months), the patients received colchicine therapy. While 24 of these patients were compliant with the treatment, 14 patients remained non-compliant. Of the 24 compliant patients, 19 had normal renal function at the onset; in 13 the proteinuria improved, in 5 patients it remained stable, and in 1 patient it deteriorated from a proteinuric to nephrotic stage. Partial resolution of amyloidosis was demonstrated by repeat renal biopsy in 1 patient who showed complete resolution of proteinuria. In contrast, none of 14 non-compliant patients improved, and while only 1 patient was in renal failure initially, 10 patients deteriorated to renal failure during the follow-up period. The presence of tubulointerstitial injury at presentation adversely affected the prognosis. In conclusion, when used appropriately, colchicine can improve proteinuria and prevent chronic renal failure in patients with amyloid nephropathy of FMF. The presence of renal failure or tubulointerstitial injury at presentation and non-compliance with therapy are the factors decreasing the success of therapy.