Children born with the rare congenital condition Freeman-Sheldon syndrome (FSS) have a characteristic facial appearance: microstomia, a long philtrum, ‘H-shaped’ chin abnormality and ‘pinched lips’ in addition to extra-facial features such as kyphoscoliosis and hand deformities. Such children often have problems with oral continence and difficulties with speech leading to both nutritional and psychosocial concerns. Prompt correction through surgery is therefore important in effectively managing the condition. We report the case of a 7-year-old girl who presented with the appearance of scarred lips with difficulties in closing her mouth, a speech impediment and oral incontinence. Using a muscle-sparing technique to reconstruct the upper and lower lips, better oral continence was achieved alongside markedly improved speech and an aesthetically pleasing result. Taken together, this case aptly illustrates the benefits of lip reconstruction in improving function and cosmesis in patients with FSS.