In most cases, the clinical manifestation of the agnogenic myeloid metaplasia is an anemic syndrome. Ascitis secondary to portal hypertension as the first manifestations is a rare clinical condition. Finally, the form of presentation of the idiopathic rare. We present a case of agnogenic myeloid metaplasia in a 64 years-old patient whose form of presentation was an spontaneous infection of the ascitic fluid. In our review of the literature we have not found any other case with similar characteristics.