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Anaplastic lymphoma kinase positive large B-cell lymphoma (ALK+LBCL) is a rare subtypeof B-cell lymphoma with aggressive features and a poor prognosis. The rarity of this diseaseand similarity of microscopic features with undifferentiated epithelial neoplasm disturb theprovision of proper treatment. A 57-year-old male presented with a rapidly growing and painlessmass on the left side of the neck. Metastatic carcinoma of an unknown primary (CUP) lesionwas initially suspected based on the pathologic results obtained from a core needle biopsy,but it was finally diagnosed as ALK+LBCL after a modified radical neck dissection. We hereinreport a case of ALK+LBCL mimicking CUP with a literature review