GLI1-altered mesenchymal tumors are a relatively rare type of mesenchymal tumor that is common in the head and neck. It is a newly added tumor type in the World Health Organization Tumor Classification of Head and Neck (5th edition). This article reports a case ofGLI1-alteredmesenchymal tumor occurring at the left tongue and reviews the relevant literature to summarize the pathological morphological characteristics, immunophenotype, molecular changes, clinical manifestations, and prognosis of the disease.