Congenital adrenal hyperplasia presenting as hematuria and acute renal faliure.

We present a neonate who presented with hematuria and acute renal failure. Classical 21 hydroxylase deficiency was diagnosed on the basis of features of salt wasting, response to treatment with corticosteroids and mineralocorticoids and a positive ACTH stimulation test. Renal vein thrombosis secondary to hemoconcentration due to salt wasting was attributed as the cause of hematuria. Follow-up revealed clinical improvement and normalization of renal parameters. This is the first report of congenital adrenal hyperplasia presenting as hematuria and renal failure to the best of our knowledge.