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Introduction: Factor VII deficiency is a rare congenital coagulopathy disorder. In most cases this disorder is diagnosed in childhood. Common symptoms of congenital factor VII deficiency are different and consist of mucosal cutaneous hemorrhage, gastrointestinal bleeding, and joint bleeding. CNS hemorrhage is a fatal and severe complication of congenital factor VII deficiency. The incidence of gross hematuria is a rare symptom of factor VII deficiency. Isolated presentation of hematuria is rarer and usually is accompanied by bleeding in other sites. Case Presentation: The patient reported here is a 6-month-old girl who was diagnosed with congenital Factor VII deficiency following episodes of isolated gross hematuria. We decided to report this case to demonstrate if there is no other organic cause in the investigation of a child with recurrent hematuria, we should also consider a coagulation factors deficiency. Conclusion: Since isolated hematuria is a rare symptom in the coagulation factors deficiency, the coagulation tests may be of less interest.