A 6-day-old Pakistani boy was admitted to hospital in June 2005 for bleeding from the left nipple. His parents are first cousins. He has two siblings aged 8 and 6 years. There was no family history of bleeding diathesis. The mother had a normal pregnancy with full antenatal care. Mode of delivery at term was an elective Caesarean section due to previous Caesarean sections. His birth weight was 2.7 kg. 1 mg of Vitamin K was given intramuscularly at delivery. His first neonatal check was normal. He had a small amount of self-limiting bleeding from the umbilical cord on day 3 of life. He went home on day 4. On day 6 of life he had mild spontaneous left sided nipple bleeding, which presented as spots of blood stains on the left side of his baby dress. Over the next 7 days he had two further episodes of a similar nature. Baseline biochemistry and full blood count were normal but a coagulation screen showed a prolonged prothrombin time of 41 s (control 14 s), a prolonged partial thromboplastin time of 132 s (control 33 s) and normal thrombin time of 15 s (control 14 s). In view of the mild nature of bleeding, the child was only treated with 1 mg of i.v. vitamin K. Subsequent coagulation assays revealed a plasma factor V activity of less than 0.01 IU/ml (normal range 0.50–1.50 IU/ml) determined by factor V clotting assay with all other coagulation factors in the normal range. Hence the diagnosis of congenital factor V deficiency was made. A cerebral ultrasound done at this stage was within normal limits. Oral transexamic acid at a dose of 15 mg/kg/dose 3 times daily was started. Mother was found to have a plasma factor V activity of 0.44 IU/ml (normal range 0.5–1.50 IU/ml) and the father of 0.52 IU/ml by factor V clotting assay. Both levels are compatible with heterozygous factor V deficiency. The siblings had factor V activities of 0.61 and 1.1 IU/ml respectively. Genetic analysis at the St. Thomas Hospital hemophilia molecular genetics laboratory showed that the affected child is homozygous for a frame shift mutation resulting in a premature termination sequence at codon 2178 in exon 25 of the factor V gene and both parents are heterozygous for this mutation. The infant presented at day 15 of age with a further episode of bleeding from the umbilical stump. The bleeding stopped promptly following administration of 20 ml/kg of fresh frozen plasma (FFP, methyleneblue sterilized, single donor and US sourced). Small bruises were noted around the venepuncture sites from the previous admission. A hematoma measuring 2 cm in diameter was noted on the anterolateral aspect of the left thigh. It was attributed to the injection of vitamin K at birth. His head circumference was increasing within normal range and a repeat cerebral ultrasound was normal. The patient presented again at 5 weeks of age with pallor, irritability, lethargy and reduced feeding. His anterior fontanelle was bulging and tense. He was hemodynamically stable. His hemoglobin was 5.6 g/dl, which represented a significant drop from the previous estimation 3 weeks back. The cerebral ultrasound was repeated and showed a large intracerebral hemorrhage extending from the frontal lobe to the parieto-occipital region. There were a few cystic areas seen within this bleed, which suggested that the bleeding started at least a few weeks ago. The child was immediately transfused with 20 ml/kg of FFP and 15 ml/kg of packed red blood cells. He had a series of generalized fits within 5 hours of admission. The patient was transferred to the regional tertiary referral centre for specialised neurosurgical and haematological management. A cranial computed tomography (CT) scan showed a right, large, intracerebral bleed causing a shift of the midline to the left. The ventricles were not enlarged and the bleed did not extend into the ventricles. A Hickman line insertion was arranged to enable regular FFP infusions. Preoperatively he was transfused again with 20 ml/kg FFP and 10 ml/kg of platelet concentrate to achieve adequate hemostasis. Platelets are known to be a good source of factor V as it is stored in the platelet alpha granules. He was also given recombinant factor VIIa (Novoseven, NovoNordisk®) as an empirical adjunctive agent in the peri-operative period in view of the fact that reliably hemostatic levels of factor V (0.25–0.30 IU/ml) are difficult to achieve in this condition. Factor VIIa is unlikely to work in the absence of any factor V but can contribute to increased thrombin formation once factor V is present. Postoperatively a regime of FFP (15 ml/kg/dose) administration was set up: FFP twice a day for the first 10 days followed by once a day for the next 10 days. This was followed by at least alternate days (every 48 hours) of FFP at 20 ml/kg/day up to the present. Plasma factor V activity has been monitored: A maximum factor V level of 0.23 IU/ml was achieved within 20 minutes after transfusion. Trough levels were between <0.01 to 0.05 IU/ml. When undetectable factor V levels were noted inhibitor assays were performed. Inhibitors have not been detected so far. No further bleeding has been noted up to the present day. Immunisations including hepatitis B vaccination were given subcutaneously as is normal practice for children with severe bleeding disorders. Follow up until the present (21 months of age) revealed an appropriate increment in head circumference. Neurodevelopment has been within normal limits.