Pseudomyogenic hemangioendothelioma (PMHE) is a rare vascular tumor that was added to the World Health Organization classification of soft tissue tumors. These tumors have a unique clinical presentation and microscopic appearance as compared to other vascular tumors in the differential diagnosis. Unlike its microscopic mimicker epithelioid sarcoma, PMHE rarely metastasizes and long-term survival in affected patients is excellent. In this report, we present a patient with PMHE and review the current literature on clinical presentation and histologic differentiation of this unique tumor, comparing findings to its mimickers.