Objective To discuss a new method of one-stage upper-lip vermilion reconstruction with a modified pedicle lower-lip compound tissue flap in progressive hemifacial atrophy. Methods From November 2014 to February 2016, 6 cases with secondary upper-lip defect caused by progressive hemifacial atrophy were treated. According to the degree and size of upper-lip vermilion, lower-lip compound flap pedicled by inferior labial artery was transposed to reconstruct the defect on upper-lip vermilion in one stage. Results All the flaps survived completely. All the patients were followed up for 3 to 12 months, satisfactory aesthetic results were achieved. The oral commissure kept normal with no obvious scar at the donor sites. Conclusion The improved method has the advantages of avoiding inconvenience in feeding, speaking and cleaning. The procedure is simple with available blood supply and short treatment duration. Both aesthetic and functional results are satisfactory.