SummaryWe report an unusual evolution of heterozygous Fabry’s disease. Progressive renal failure occurred in a 45-year-old woman. Endstage renal failure in the fourties is usually observed in hemizygous male patients with Fabry’s disease but is unexpected in heterozygous females. In the present case, tuberculosis induced unilateral hydronephrosis was also found and could have precipitated renal failure.The association between Fabry’s disease and renal tuberculosis has been previously reported. Nevertheless a definitive relationship between both conditions is still not demonstrated.