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Epithelioid hemangioma is a rare tumor that can have bone involvement. Its clinically and radiographically aggressive appearance mimics a malignant neoplasm. Although epitheliod hemangioma has been described as having an aggressive appearance on magnetic resonance imaging (MRI) and plain radiographs, this is the first reported case of pathologic fracture associated with this lesion to our knowledge. This article describes a case of epithelioid hemangioma involving the distal humerus, which initially presented with progressive pain and fracture of the lateral condyle. The aggressive appearance on plain radiographs and MRI suggested a malignant bone tumor. This preliminary diagnosis was confirmed due to the presence of local lymph node spread on positron emission tomography/computed tomography. After a core needle biopsy revealed nondiagnostic tissue, rather than performing a wide resection based on a presumptive malignant diagnosis, we followed the standard diagnostic algorithm and performed an open biopsy with temporary internal stabilization. The tissue sample was adequate and revealed a diagnosis of epithelioid hemangioma. Based on this finding, we were able to proceed with surgical management, including curettage of the lesion, placement of a bone graft, and internal fixation, rather than a wide resection with elbow joint replacement. This article emphasizes the need for careful adherence to the diagnostic algorithm for musculoskeletal tumors. In doing so, a definitive diagnosis was reached, and our patient was able to resume his occupation as a laborer without the restrictions that would have accompanied elbow arthroplasty. [ABSTRACT FROM AUTHOR]