We report a case of a leiomyomatous hamartoma located in the midline of the base of the tongue. The two-year-old girl had an asymptomatic pedunculated tubular tumour measuring 1.6 x 1.3 x 1.1 cms. The lesion was surgically excised, and histopathological examination disclosed a non-neoplastic mass composed of irregular arranged bundles of smooth muscle cells embedded in a fibrovascular stroma. The diagnosis was confirmed by immunohistochemistry with positivity for smooth muscle actin and desmin. Until now, only 19 cases have been reported in English-language literature.
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