While local anesthesia remains standard for muscle biopsies, it often inadequately controls procedural pain. General and spinal anesthesia carry risks in neuromuscular disorders. Ultrasound-guided nerve block may reduce the risk of anesthetic complications and pain during muscle biopsy. We aimed to evaluate the safety and analgesic efficacy of ultrasound-guided nerve block during muscle biopsy procedures. We analyzed ten consecutive cases undergoing muscle biopsy with ultrasound-guided nerve block from April 2022 to April 2024. Data included biopsy site, nerve block location, anesthetic agent type, anesthetic dose, procedure timeline (block-to-biopsy interval, biopsy duration), pain assessment, and anesthetic complications. Brachial plexus block was selected for 6 patients with biceps brachii biopsy, and femoral nerve block for 4 patients with quadriceps biopsy. Anesthetics were 1% lidocaine (Xylocaine) in 9/10 patients. The mean time from block to biopsy was 15.0 minutes. No complications such as nerve injury, vascular injury, or infection occurred. Only 2/10 patients reported pain during muscle biopsy (Visual Analog Scale (VAS) scores: 3/10 and 4/10). Ultrasound-guided nerve block appears safe and effective for pain control during muscle biopsy, with 80% complete analgesia and no adverse events. Study limitations include a small sample size; larger trials are needed to establish optimal protocols.