A 56-year-old male with distal myopathy of rimmed vacuoles underwent laparoscopic nephrectomy. Anesthesia was induced with propofol, remifentanil and ketamine. Tracheal intubation using McGRATH was uneventful without using muscle relaxants. Then ultrasound-guided right thoracic paravertebral (TPVB) block was performed using 20 ml 0.75% ropivacaine with 10 ml 2% lidocaine by 3 injections of 10 ml each at T9 to T11. General anesthesia was maintained with propofol, remifentanil and ketamine monitoring bispectral index. Good surgical condition and pneumoperitoneum were maintained without using muscle relaxants. His postoperative course was smooth and uneventful, even though a small amount of fentanyl was administrated to relieve wound pain. This case suggests that McGRATH and ultrasound-guided TPVB can be one of the options to avoid using muscle relaxants in patient with neuromuscular disease.