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Hematological conditions rarely present as isolated hematuria. Factor VII deficiency is a rare congenital coagulopathy inherited as autosomal recessive pattern. It usually presents a severe life-threatening bleeding at an early age. The presentation of congenital factor VII factor deficiency for the first time as recurrent hematuria at a later age is a rare presentation. We report a case of a 23-year-old male, who presented to us with recurrent episodes of painless hematuria for the past three months due to congenital factor VII deficiency.