Introduction: Congenital afibrinogenemia is a very rare hereditary anomaly of coagulation. Only 150 cases have been published. Clinical manifestation in the form of some type of bleeding is similar to that of other congenital coagulopathies, although the pattern of presentation is different. Spontaneous bleeding is rare, but slight injury, which may be unnoticed, may trigger it off. In spite of being a congenital condition, it may be of late onset, as in our patient, with bleeding episodes occurring in the second decade of life.
Clinical case: We describe a woman who had several episodes of bleeding, two of which were intracerebral. The principal feature of this was dissociation between the clinical findings and their detection by neuro-imaging. Substitutive therapy led to the disappearance of symptoms.
Conclusion: Cerebral haemorrhage in the presence of afibrinogenemia may fail to be detected early on CT. On clinical suspicion of bleeding, early substitutive treatment should be started.