Familial juvenile hyperuricemic nephropathy (FJHN) is an autosomal dominant renal disease characterized by juvenile onset of hyperuricemia, gouty artritis, and progressive renal failure at an early age. Early treatment with allopurinol may prevents renal deterioration. Symptomless members of families in which the condition has been identified should be screened also, for renal function and urate handling. Here we report two brothers with FJHN which present after the development of renal failure.