Background <p>Factor XIII (FXIII) deficiency is a rare autosomal recessive coagulation disorder characterized by impaired fibrin stabilization and delayed bleeding despite normal routine coagulation parameters. Intracranial hemorrhage represents its most severe and life-threatening manifestation, particularly in the infant population.</p> Case description <p>We report a 5-month-old female infant presenting with a right frontal lesion following minor trauma, initially suggestive of tumor or abscess based on its radiological appearance. The patient underwent craniotomy; however, recurrent postoperative hematomas developed despite consistently normal routine coagulation tests. Further targeted hematological evaluation revealed markedly reduced FXIII activity (15%, reference range 70–140%), confirming the diagnosis of FXIII deficiency. Following initiation of replacement therapy with fresh frozen plasma, no further hemorrhagic episodes occurred.</p> Conclusion <p>Factor XIII deficiency should be considered in infants presenting with recurrent intracranial hemorrhage and normal routine coagulation tests, particularly in the presence of clinical risk factors such as neonatal umbilical bleeding and parental consanguinity. Early targeted laboratory evaluation and prompt initiation of replacement therapy may prevent repeated surgical interventions and improve clinical outcomes.</p>