Intrahepatic lesions in adults, commonly named hepatic hemangioma, should be called Intrahepatic Venous Malformations (IHVM), or Giant Intrahepatic Venous Malformations (GIHVM) when larger than 10 cm according to the ISSVA classification (International society study group for vascular anomalies). Localized coagulation disorders (LIC) in patients with venous malformations are quite commonly associated in venous malformations, they result in decreased fibrinogen (< 2g/l) and elevated d-dimers (> 1500 ng/ml) and might be responsible of intralesional thrombotic, pain or bleeding episodes.We report a case report of a 41 y/o patient that presented with right hypochondrium pain episodes discovering an unknown GIHVM on ultrasound imaging with a prior history of uterine bleeding episodes and multiples miscarriages.On laboratory work up the patient presented an associated localized Intravascular Coagulation (LIC) with the GIHVM. As the patient desire to become pregnant was important our multidisciplinary clinic allowed a pregnancy with close clinical, biological and imaging monitoring and follow up. Early initiation of low molecular weighted heparin (LMWH) successfully allowed an uncomplicated term pregnancy and delivery. Intrahepatic lesion stability was achieved and prevented progression from LIC to diffuse intravascular coagulation disorder (DIC)..