ACE and X-linked hydrocephalus with stenosis of the aqueduct of Sylvius: Currently, the patient is being treated with an angiotensin-converting enzyme inhibitor (ACEI).<h4>Conclusion</h4>Early renal biopsy and comprehensive genetic analysis in a child with a positive family history of renal disease allowed the expedited diagnosis of X-linked Alport syndrome (XLAS), after the detection of persistent microscopic hematuria in 20 days.