Ongoing thrombosis in the distal portal veins in our patient is sustained by different observations: First, our patient presented with a low level of protein S during the thrombophilia workup pointing to a coagulation disorder that has been found in the majority of patients presenting with NRH (protein S or C deficiency and/or anti-cardiolipin antibodies) [10,14]. This evidence concerns the gene PROS1 and Rare hereditary thrombophilia.