The authors report on the case of a 32-year-old woman with an intramuscular arteriovenous hemangioma (AVH) of the left forearm with burning pain and paresthesias diffused to the radial nerve-related territories. The patient underwent coil embolization of the AVH and surgical removal of the remnant and regrown AVH. This case demonstrates the safety and efficacy of surgery when interventional radiology fails to achieve complete occlusion. En bloc removal of the lesion was performed through a left elbow cleft incision, and intraoperative electrophysiological monitoring and angiography with indocyanine green (ICG) were performed. The pathological diagnosis was intramuscular AVH. Postoperative follow-up examinations demonstrated the permanent disappearance of the subcutaneous mass and of the patient's sensory disturbances. Complete excision of the AVH was confirmed on postoperative magnetic resonance angiography, and no surgery-related complications or new neurological symptoms were detected. Intramuscular AVHs are rare lesions that can be successfully treated with both coil endovascular embolization and surgery; the latter is indicated when endovascular procedures fail to occlude the AVH completely. Intraoperative angiography with ICG can be helpful in confirming the success of the procedure.