Duplication of renal collecting system in association with neurofibromatosis type 1

Duplication of renalcollecting system in neurofibromatosis type 1 NF1 is reported. The patient, the son of a mother with NF 1, was 10 months old who presented with acute febrile respiratory distress. In addition there were significant cafe' au-lait spots on the trunk and freckles in right axilla,macrocephaly,pectus excavatum, pulmonary stenosis and right palpable kidney, findings consistent with the diagnosis of NF1.To the best of our knowledge such association. which may have genetic basis, lias not been previously reported