Introduction: Neurofibromatosis type-I (von Recklinghausen's disease) is characterised by multiple cutan neurofibromas, cafe-au-lait spots, bone abnormalities and tumours of central and peripheral nerves. Gastrointestinal involvement occurs in 25 percent of all cases.
Case report: The authors report a case of a 53-year-old man presenting with rare hepatobiliary complication. He complained of abdominal pain and he looked jaundiced. The abdominal ultrasound revealed cholestasis confirmed by the laboratory results. The endoscopic retrograd cholangiography showed nearly total obstruction in the middle part of common bile duct. Subsequently laparotomy and choledochoenterostomy was performed. Plexiform neurofibroma was found in the wall of common bile duct and several gastrointestinal stromal tumour seedings were identified on the serosal surface of the small intestine. Post surgery he remained symptom free and showed no sign of jaundice.
Conclusion: The authors would like to draw attention to this extremely rare case, since only two similar cases appeared in the literature.