A fibrinogenemiais a rare autosomalrecessivediseasewhoseclinical manifestations are early and of variable severity, rangingfrom minimal bleeding to cataclysmichaemorrhage. In some cases, thromboembolic complications mayappear, depending on the severity of the disease.We report here the observation of a young patient, followedsincechildhood for a congenitalafibrinogenemiahaving been hospitalizedseveral times for haemorrhagic,cutaneous and ENT manifestations ,who has been admittedinto an array of intracranial hypertension secondary to cerebralhaemorrhagewithuppergastrointestinalbleeding. Endoscopic exploration performedremotely from the haemorrhagicepisode, aftercorrecting the fibrinogenlevel, revealedesophageal varices with no redsignswith a gastric one classified as GOVII. As far as the etiologicalassessment of portal hypertension (PHT) isconcerned, an abdominal ultrasoundwasperformedobjectifying a portal cavernomawith PHT signs. Takingintoconsiderationboth of the risk of haemorrhage and the remoterealization of endoscopy, the ligation of oesophageal varices was not performed. The patient was put underbeta-blocker, withoutrecurrentbleedingafter a follow-up of two and a halfyears.Based on this observation, werecall the clinicalparticularities of such a rare condition as well as the treatmentmodalities.