OBJECTIVE To evaluate the efficacy of the combined approach of preoperative endovascular embolization (EE) and surgical excision (SE) for scalp arteriovenous malformation (AVM) and present an illustrative case report. METHODS A systematic review was conducted using online databases (PubMed/Medline, Cochrane, and Embase) on February 15, 2023. The inclusion criteria were any type of study of patients with scalp AVMs who were diagnosed and confirmed through angiography and treated with combined preoperative EE and SE. All the articles that met the inclusion criteria were included in this study. RESULTS A total of 49 articles (91 patients) were included. The patients' age ranged from 10 days to 70 years at the time of presentation. The most common symptoms were a pulsatile mass in 51 patients (56.04%), progressively growing mass in 31 patients (34.06%), and bruits and/or thrills in 22 patients (24.17%). Complications of preoperative EE and SE were observed in only 5 patients; 3 patients (3.29%) had harvested skin graft marginal necrosis, 1 patient (1.09%) had skin necrosis, and 1 patient (1.09%) had a wound infection. Only 2 patients (2.19%) reported a recurrent or residual mass during a median follow-up period of 12 months. CONCLUSIONS The management of scalp AVMs can be challenging; therefore, focused, and accurate identification of the complexity of the vascular anatomy is required. The combined method of preoperative EE and SE showed satisfactory outcomes with low rates of complications and recurrence; thus, we recommend this approach for the management of scalp AVMs.