A 32-year old woman presented with progressively increasing aundice, intractable pruritus and pale stools for 2 months. There as no fever, prodromal symptoms, abdominal pain, vomiting or astrointestinal bleeding. She had also noticed a rapid increase n abdominal girth over the last month. Examination of her skin Fig. 1A) showed brownish hyperpigmented macules and subcuaneous nodules suggestive of neurofibromatosis type 1 (NF1). he was deeply icteric with moderate ascites. Ultrasound of the bdomen showed a dilated common bile duct, intrahepatic biliary adicles and ascites. Side viewing endoscopy demonstrated a perimpullary growth (Fig. 1B) with histology comprising of intestinal ucosa with adjacent malignant glands infiltrating the underlyng submucosa and muscularis consistent with adenocarcinoma Fig. 2A and B). Ascitic fluid cytology was positive for malignant ells.