Introduction This study aimed to illustrate the efficacy of the combination of lens extraction, trabeculectomy, and anterior vitrectomy in patients with secondary angle-closure glaucoma (ACG) with autosomal recessive bestrophinopathy or Best vitelliform macular dystrophy. Methods This is a retrospective self-controlled case series study. Five patients undergoing a single trabeculectomy in one eye and triple surgery in the other eye were enrolled. All patients underwent a complete ophthalmic examination that included best-corrected visual acuity (BCVA), intraocular pressure (IOP), ultrasound biomicroscopy, and static gonioscopy. Multimodal fundus imaging was performed, including color fundus photography, fundus autofluorescence, and optical coherence tomography. Genetic testing was also analyzed. Results Among the 10 eyes, the mean IOP was 31.4 +/- 4.7 mmHg before surgery. The mean axial length (AL) was 21.53 mm and the anterior chamber depth (ACD) was 2.31 mm. There were no statistically significant differences in preoperative IOP, BCVA, ACD, and AL between the two groups (all P > 0.05). The mean follow-up time was 64.0 months. All five eyes with a single trabeculectomy developed malignant glaucoma (MG). No complications were found in the other five eyes with triple surgery, and the anterior chamber was deepened and stable after surgery until the last visit. The mean IOP at the last visit was normalized to 16 mmHg without using any medications. Conclusions Triple surgery is superior to single trabeculectomy for patients with ACG and BEST1 mutation, effectively bypassing MG complications. The vitreous may play a vital role in the mechanism of ACG in those patients and the high incidence of MG after filtering surgery.