Sacras, MLLadeira, CCarmo, SKjöllerström, P2022-09-142022-09-142021Port J Pediatr 2021;52:193-6http://hdl.handle.net/10400.17/4247Hemophilia A, characterized by deficiency of functional plasma clotting factor VIII, is an X-linked disorder. Signs and symptoms depend on factor VIII levels. Severe hemophilia A (factor levels less than 1%) is usually diagnosed in the first two years of life. Pediatric compartment syndrome is most associated with trauma, but infectious and vascular causes must also be considered. We report a case of a newborn who presented with a severe hand hematoma after venous puncture, complicated by compartment syndrome. An isolated prolonged partial thromboplastin time was found, and hemophilia was suspected. Factor VIII levels less than 1% were consistent with severe hemophilia A. The patient received recombinant factor VIII perioperatively. Fasciotomy was performed and the patient was kept on antibiotics until closure.engAcute DiseaseCompartment Syndromes/ diagnosisCompartment Syndromes/etiologyCompartment Syndromes/therapyFasciotomy; Hemophilia A/complicationsHemophilia A/diagnosisInfant, NewbornCase ReportHDE CIR PEDHDE HEM PEDjournal articledoi.org/10.25754/pjp.2021.20470