RESUMO
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown etiology, probably immune-mediated. PSC is frequently associated with Inflammatory Bowel Disease, usually Ulcerative Colitis and less commonly with Crohn's disease. The small-duct PSC variant occurs in 5%of patients. Eosinophilic gastroenteritis (EG) is another chronic inflammatory disease, characterized by eosinophilic infiltration limited to the digestive tract, and probably of immunoallergic origin. EG is frequently observed in children but it's less commonly seen in adults. EG can affect any segment of the gastrointestinal tract, and recently it has been described an increase in the incidence of the esophagic variant, termed eosinophilic esophagitis.Ileocolonic involvement in EG is rare and clinical manifestations depend of the intestinal layer affected. Patients with mucosal infiltration complain of abdominal pain, fecal occult blood loss and/or protein-losing enteropaty, while signs and symptoms of obstruction are common in those with muscular EG, finally involvement of the serosal layer occurs in 10% and typically presents as eosinophil-rich ascitis. Response to steroids usually is excellent. There is a previous publication in the literature documenting the association of PSC and EG. Here we describe the first case of small-duct PSC associated to EG with ileocolonic involvement.