This is the case of a female patient aged 42, admitted for abdominal distension and abdominal pain, in whom clinical examination revealed a dysmorphic liver with signs of portal hypertension. Abdominal ultrasound coupled with Doppler showed SBC, which was confirmed by abdominal angioscan. A complete etiological workup was performed, which revealed the presence of an HPN clone on flow cytometry. The patient was put on anticoagulation in addition to treatment of the complication of PH, with a good clinical evolution. At present, the patient is still under regular consultation.