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Primary left hepatic duct neuroendocrine tumors are extremely rare. We describe 2 cases of siblings, a 51-year-old brother and a 48-year-old sister, who were both diagnosed with primary left hepatic duct neuroendocrine tumor. Both patients underwent successful left hepatectomy and are both alive with no recurrence. For this rare malignancy, while definitive diagnosis is made only by histopathology, a margin-free surgical resection remains the only curative treatment modality to date.

Siblings Diagnosed With Primary Neuroendocrine Tumor of the Left Hepatic Duct