Dear Sir,A 2-month-old, 4.5 kg, infant was admitted with irritability, refusal of feeds, and seizures for 3 days with an altered sensorium, bulging anterior fontanel, right limb weakness, and pallor. There was no history of fever, trauma or drug use; his maternal grandfather had hemophilia B. Computed tomography of the head showed a left parietal subdural hematoma with the midline shift; cerebral angiography was normal. Hematology revealed a hemoglobin (Hb) of 4 g%, prolonged activated partial thromboplastin time (aPTT) of 120 (32.6) sec, low-plasma factor IX (FIX) activity of 4.3%, adequate platelets and normal bleeding, clotting and prothrombin times. Hemophilia B was diagnosed and 400 international units (IU) of FIX and 50 ml of packed red cells (PRC) were administered; his Hb and aPTT improved to 9.7 g% and 30/28 sec, respectively. Emergency craniotomy and hematoma evacuation was undertaken; 400 IU of FIX was repeated half-an-hour before surgery. The operation was meticulously performed to minimize surgical blood loss; extra care was taken to prevent bleeding and hematoma formation at non-operative sites that included, gentle oropharyngeal and tracheal suctioning, adequate pressure point padding and avoiding intramuscular injections. The child had an uneventful intraoperative course; blood loss averaged 50 ml and 50 ml PRC and 50 ml fresh frozen plasma (FFP) were transfused. Postoperatively, he was ventilated overnight and intubated uneventfully the following day; FIX (200 IU/day) was administered for two more weeks raising his plasma FIX activity to 60%. He improved neurologically and had no re-bleeding.Hemophilia B is an X-linked, recessive coagulation disorder secondary to deficiency or defect of FIX; it affects 1:25,000 males and accounts for 15% of all hemophilia cases. Patients with mild (FIX ~5-40%) and moderate disease (FIX ~1-5%) have excessive bleeding following minor trauma, while severe hemophiliacs (FIX < 1%) often manifest with spontaneous hemorrhages.[1] Intracranial hemorrhages (ICH) are known (incidence ~2.2-7.8%), and are among the commonest causes of death in hemophiliacs (mortality ~34%);[23] ICH may be the first manifestation of hemophilia,[24] also seen in our patient.Surgery for ICH in hemophiliacs is a high-risk procedure though use of perioperative clotting factor has markedly improved results.[5] Preoperative normalization of FIX activity up to 60-80%, and its postoperative maintenance to over 30% for at least 14 days is recommended.[1] Factor doses (IU) are calculated by the desired rise in factor activity (%) × body weight (kg), and once daily dose of FIX is adequate; monitoring is by FIX assays or serial aPTT (acceptable values ~patients’ aPTT/normal ≤1.2).Thus a safe surgical outcome in hemophiliacs with ICH is possible with perioperative clotting factor and FFP therapy besides the routine precautions.