Parry-Romberg syndrome or progressive facial hemiatrophy is a rare clinical entity of an unknown etiology. We present the case of a 57-year-old Chinese woman with Parry-Romberg syndrome and hemifacial spasm both on the right side. Its association was not reported before. In our report of Parry-Romberg syndrome with hemifacial spasm, we try to explore whether there were some relations between the 2 diseases. We found an offending vessel compressing the root exit zone of the facial nerve and no evidence of vessel compression at ipsilateral trigeminal nerve motor rootlet.