Abstract
There is a wide variety of inflammatory and benign neoplastic disorders of the biliary system that mimic cholangiocarcinoma in terms of clinical manifestations and imaging findings. Inflammatory myofibroblastic tumour of the bilary tract is one such condition, which is extremely rare but benign. Like cholangiocarcinoma this condition presents as painless progressive obstructive jaundice and it is often difficult to differentiate between the two prior to laparotomy, with the usual investigative modalities. Diagnosis is usually established by the characteristic histopathology findings in biopsy specimen. Newer diagnostic modalities directed at obtaining preoperative biopsy of the lesion appear promising in differentiating benign from malignant biliary lesions, but their routine use is yet to become standardised. Until then, awareness of doctors about the existence of such benign entities might prompt a less aggressive treatment approach while dealing with atypical hilar lesions of liver.