Scalp hemangiomatosis, particularly large size scalp hemangiomatosis, are very rare and difficult to treat. The present report presents one case of large scalp hemangiomatosis. The patient is a 37 year-old female. The mass on the left parietal region of the head had existed for 14 years, gradually increased in area, and exhibited pulsation. Arteriovenous malformation was considered before surgery. After ultrasound and digital subtraction angiography (DSA) evaluation, the main feeding arteries were ligated. Sutures were placed around the mass to tightly clamp and control the blood supply. The mass was found to be rich in adipose tissue and blood vessels during surgery, and a pathological examination confirmed the presence of a hemangiomatosis. Postoperative scalp healing was poor, and scalp defects occurred. Local dressing change and iodoform gauze packing in the defect area resulted in complete scalp healing. The present case demonstrates that the scalp can exhibit large-size hemangiomatosis, which can be easily misdiagnosed as vascular malformations. Sufficient preoperative evaluations, including necessary examinations by ultrasound and DSA, are required to treat this disease. The feeding arteries should be clearly identified and ligated; suturing the tissues around the lesion is also effective for controlling blood loss during surgery. If possible, the lesions should be completely excised to expose the healthy scalp. If scalp defects occur, Local dressing change and iodoform strip packing can effectively promote scalp healing.