This article aims to highlight the occurrence of oral lichen planus in a patient with achondroplasia. The patient was treated with topical and systemic corticosteroids and showed significant improvement. Achondroplasia is the most common form of nonlethal skeletal dysplasia that manifests with stunted stature and disproportionate limb shortening. Clinical manifestations are mainly a result of mutations in fibroblast growth factor receptor-3 (FGFR3) gene. It is of special interest in the dental field because of its craniofacial features and dentists treating patients with achondroplasia should be well aware of the clinical features, risk factors and complications associated with this condition.