Abstract Purpose : A case presentation of a soft tissue lesion in an 8-month-old patient's left lateral tongue and review of the literature regarding infant tongue lesions. Methods : The patient's medical records were reviewed with summarization of care to date that care was received at presenting institution. A systematic literature review was then conducted using PedMed. Results : The differential diagnosis of infant tongue lesions is large, encompassing hemangiomas, lymphangiomas, thyroglossal duct cysts, teratomas, choristomas, infantile fibromatosis and rhabdomyosarcomas, among many others. Conclusion : Most infant tongue lesions are benign. However, given the aggressive propensity of infantile fibromatosis and rhabdomyosarcomas, all infant tongue lesions must be characterized.