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Obstructive jaundice secondary to common bile duct stricture is attributed most commonly to pancreatic cancer and cholangiocarcinoma. Benign tumors of the extrahepatic biliary duct such as papilloma or adenoma are rare, accounting for 6% of biliary tumors.1,2 Among them, neurofibromas of the extrahepatic biliary tract are extremely rare and clinically nonspecific. Their preoperative diagnosis is most difficult, especially when there is no history of cholecystectomy or biliary tract trauma.3,4 We report a case of such a lesion in a young female patient. Case ReportA 28-year-old female patient was referred for upper abdominal pain and fever (38.5°C) that had been evolving over eight days. She had no family history of a hereditary disorder, nor personal previous history of cholecystectomy or trauma of the bile duct. The physical examination was normal, except for tenderness in the right hypochondrium. There was no evidence of jaundice. The laboratory studies revealed: serum alanine aminotransferase (ALAT), 54Abdominal ultrasonography showed mild hepatomegaly and slight dilatation of the intrahepatic biliary tracts, which seemed to be secondary to adenopathies of the hepatic hilum measuring 15.8 mm and 12.9 mm. Abdominal computed axial tomography (CT) (Figure 1) showed a well-demarcated, homogeneous and hypodense mass (20-35 UH) on unenhanced scans, with no enhancement after injection of the contrast medium. The mass surrounded the entire common bile duct and invaded the hepatic hilum, without any dilatation of the intrahepatic biliary tracts and without adenopathy. Abdominal magnetic resonance imaging (MRI) (Figure 2) showed a tissue mass with very clear borders, infiltrating the hepatic hilum. This mass was pushing the common bile duct back, sheathing the right and particularly the left portal pedicles as well as the bile duct, with no dilatation of the intrahepatic bile ducts and no mass effect on the blood vessels. This lesion had low signal intensity on T1-weighted images with moderate and heterogeneous signal intensity on T2-weighted images: it seemed to contain more hyperintense lesions on T2-weighted images. The contrast-enhanced T1-weighted images showed no enhancement. The endoscopic ultrasound (Figure 3) revealed multiple, oval, coalescent masses at the hepatic pedicle, particularly in front of and behind the portal vein; the largest of these masses had an axis of 3 cm. No fine needle aspiration was carried out on these lesions.Evolution of pain and fever was spontaneously favorable without any treatment, especially antibiotics. There was no explanation of the fever after physical and laboratory examinations. The endoscopic ultrasound ruled out any stones in the gall bladder and common bile duct, which could explain such a high fever. Endoscopic retrograde cholangiography was not performed.Assuming that a low-grade lymphoma, sarcoidosis, an inflammatory pseudotumor of the ganglion, or ganglionic tuberculosis was involved, we performed a surgical biopsy under laparoscopy and finally diagnosed a pri...

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