Objective: Primary neuroendocrine tumors (NET) of the common hepatic duct are rare cause of malignant obstructive jaundice. Due to lack of specific features and inability to differentiate from hilar cholangiocarcinoma, they are diagnosed on postoperative histopathology. These tumors are potentially curable and usually require less aggressive surgical resection with good outcome.

Patient and Methods: A 16-year-old boy presented with abdominal pain and intermittent jaundice. On cross-sectional imaging, a localized well-defined mass was detected in the common hepatic duct with associated locoregional lymphadenopathy.

Results: Patient underwent resection of the extra hepatic biliary tract with lymphadenectomy. Histopathology revealed moderately differentiated grade 2 NET, with positive immunohistochemical markers like Synaptophysin and Chromogranin. Targeted imaging with somatostatin receptor scintigraphy ruled out residual and metastatic disease. No adjuvant therapy administered, and kept on surveillance. He was disease-free at one-year follow-up.

Conclusions: Primary NET of the biliary tract is usually a postoperative diagnosis. If suspected preoperatively, evaluation with targeted imaging and limited, organ-preserving surgical excision yields good locoregional disease control along with satisfactory long-term outcomes.