We present a case of basal cell adenocarcinoma (BCAC) in the tongue in a 65-year old male. This is an extremely rare presentation. BCAC generally occurs in the parotid gland and rarely involves the minor salivary glands. Few cases have been reported in literature with a variable presentation. The biopsy was formalin-fixed and paraffin-embedded. The sections were stained with routine Hematoxylin and Eosin. Immunohistochemistry was performed. Hematoxylin and eosin staining showed tumour composed of variable sized and shaped, nests and sheets of basaloid epithelial cells having hyperchromatic to vesicular nuclei. Immunohistochemistry was positive for Pancytokeratin, Epithelial membrane antigen and p53. The clinicopathological features and the cellular immunophenotype addressed the diagnosis towards BCAC of the tongue. The goal of this report is to increase awareness of this rare disease and to review and discuss the differential diagnosis and important considerations in treatment.

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