A case of a 3-year-old boy with severe haemophilia A who had a successful neurosurgical drainage of a combined spontaneous left temporal subdural and intra-parenchimal haematoma is reported. Surgical intervention was required because of clinical worsening during conservative treatment with dexamethasone and factor VIII (FVIII) replacement therapy. Continuous FVIII infusion was given before, during and after the procedure. There were no surgical complications and neurological examination remains intact. Neurosurgical interventions may be reserved for special, high-risk cases, as the one presented.