Lumbar disc herniation (LDH) in patients with hemophilia A (HA) presents significant surgical challenges due to elevated perioperative bleeding risks. Traditional surgical approaches may increase the likelihood of complications such as epidural hematoma and disc reherniation, necessitating innovative strategies. This report introduces arthroscopic-assisted uniportal spinal surgery (AUSS) combined with annular suturing repair and ligamentum flavum preservation as a minimally invasive approach designed to mitigate these risks and improve surgical outcomes in patients with HA.A 20-year-old male presented with a 1-year history of lower back pain and 4 months of right leg pain and numbness, worsened by standing and walking. Magnetic resonance imaging and computed tomography revealed L5/S1 disc herniation compressing the right nerve root. The patient's history of HA extended over 19 years.LDH at L5/S1 and HA.The patient underwent an AUSS with annular suture repair and ligamentum flavum suspension. Intraoperatively, the herniated nucleus pulposus was excised, and the annular defect was sutured to mitigate reherniation risk. Perioperative management included factor VIII replacement to stabilize the coagulation levels.Postoperatively, the patient experienced significant relief from symptoms. Follow-up magnetic resonance imaging at 1 and 6 months showed no recurrence of the disc herniation. The patient returned to normal activity without any complications.This case illustrates that AUSS with annular suturing repair is a feasible and effective approach for treating LDH patients with hemophilia, offering minimal bleeding risk, and reduced recurrence of disc herniation.Copyright © 2025 the Author(s). Published by Wolters Kluwer Health, Inc.