Renal involvement in acro-renal-ocular syndrome: interstitial nephritis, unlikely pyelonephritis

We describe a case, seemingly sporadic, in which ocular and acral malformations were associated with an interstitial nephropathy; we made a diagnosis of acro-renal-ocular syndrome. There was no stasis, reflux, or urinary tract infection in our patient. We believe that interstitial nephritis in acro-renal-ocular syndrome may not always be pyelonephritis, as previously reported.