Composite hemangioendothelioma (CHE) is a rare and little-known condition with combined benign, intermediate, and malignant features. In the current study, we describe the case of a young female with the presentations of CHE, representing the second known case in Iran. Also, we have comprehensively reviewed previous case reports of CHE. A 30-year-old female was referred with a reddish hemorrhagic painless mass in the small right finger that appeared within a few days following trauma. The mass was primarily excised but recurred within three weeks; therefore, she underwent thorough clinical, laboratory, and imaging studies. Finally, the lesions were biopsied and diagnosed as CHE. Accordingly, the involved finger was amputated, and the patient underwent chemoradiotherapy. Although CHE is a rare malignant condition worldwide, attention to the clinical presentations of this malignancy can help scientists make better therapeutic approaches leading to the best outcomes.