Abstract. Hemangioma is a common disease; however, intramuscular hemangioma (IH) presenting with a pathological fracture is extremely rare. The present study reports a case of a 46-year-old male patient that suffered from IH of the right thigh, presenting with a pathological femoral fracture. The patient was initially diagnosed with a traumatic femoral fracture, and routine open reduction and internal fixation were performed at a local hospital. However, 20 days subsequent to surgery, gradual swelling and soreness around the incision were observed. The incision eventually ruptured during squatting for bowel movement, which led to extensive blood loss. Based on computed tomography (CT) and deep femoral artery arteriography, IH presenting with a pathological femoral fracture was diagnosed. The patient underwent artery embolization, from which he recovered well. At the 6-month follow-up, the femoral fracture was revealed to have healed, and a CT scan demonstrated no evidence of recurrence; however, continuous observation using CT is required in order to determine the long-term outcome. To the best of our knowledge, this is the first case of a misdiagnosed pathological femoral fracture in a patient with IH reported in the English literature.