Background: Composite hemangioendothelioma (CHE) is a rare and enigmatic vascular neoplasm that exhibits complex and heterogeneous characteristics. This comprehensive review explores the unique nature of Composite hemangioendothelioma (CHE), with a particular focus on its occurrence at the base of the tongue. Composite hemangioendothelioma (CHE) falls within the category of locally aggressive, rarely metastasizing vascular neoplasms. It is characterized by the presence of histologically distinct components, demonstrating features of benign hemangioma and malignant angiosarcoma. A thorough literature review of pubmed published articles was done. Case details: This article presents the first-ever case report of CHE involving the base of the tongue, contributing to the limited existing knowledge in this field. Accurate diagnosis and differentiation from morphological mimics are essential to determine prognosis. Surgical resection with negative margins is the primary treatment modality, while radiotherapy may be considered in certain cases. Conclusion: Long-term surveillance is necessary due to the potential for multiple recurrences and lymph node metastasis.