A 75-year-old woman with known systemic mastocytosis presented with ab
dominal pain, ascites, and bi re duct thickening on computed tomograph
y and ultrasonography. A liver biopsy specimen showed infiltration wit
h mast cells, Endoscopic retrograde cholangiography showed ductal chan
ges compatible with those found in primary sclerosing cholangitis. Bru
sh cytology of the intrahepatic bile ducts confirmed mast cell infiltr
ation, Systemic mastocytosis can infiltrate the biliary system, produc
ing a cholangiopathy radiographically similar to primary sclerosing ch
olangitis.