A 35-year-old male diagnosed with Wegener's granulomatosis (WG) in 1989 recently came to our hospital with the complaint of left lumbar pain. He was found to have left nephrolithiasis and left diaphragmatic elevation. Fluoroscopic study and electromyographic examination revealed findings compatible with unilateral phrenic paralysis. We could not detect any cause other than WG which could be responsible for the unilateral diaphragmatic paralysis. Although different types of lung involvement have been reported in WG, to our knowledge this is the first in which unilateral phrenic nerve paralysis and diaphragmatic elevation associated with WG have been diagnosed.