Soft tissue myoepitheliomas, in contrast to salivary gland myoepitheliomas, are benign neoplasms that typically occur in the soft tissues of the extremities. Both are characterized by a multilobular proliferation of polygonal to fusiform cells embedded in a variably myxoid to chondromyxoid matrix. A histologically similar lesion that has a marked predilection for the anterior dorsum of the tongue has been referred to as ectomesenchymal chondromyxoid tumor. The morphological and immunohistochemical resemblance of soft tissue myoepitheliomas to ectomesenchymal chondromyxoid tumors has led to the use of these designations interchangeably. We present a case of myoepithelioma of the tongue and review the literature, with emphasis on the differential diagnosis and histogenesis of this lesion and pertinent nosologic considerations.