Achondroplasia is the most common skeletal dysplasia caused by a mutation in the fibroblast growth factor receptor, type 3 gene (FGFR3) and resultant disabling growth and developmental disorders. This complex condition often presents with significant general and specific manifestations. Its oral consequences involve dental formation anomalies and early loss of teeth. Facing a situation of edentulism, careful considerations must be taken into account in prosthetic management depending on the condition of the osteomucosal bearing surfaces and the patient’s aesthetic context and preferences, both affected by the specific implications of the syndrome. Accordingly, the treatment protocol must be meticulously tailored to address these distinct considerations, and approaches to treatment must be effectively adjusted to meet the specific needs when taking impressions and managing prosthetic space and aesthetic results by selecting and mounting prosthetic teeth to achieve optimal functional and aesthetic outcomes. This case report describes the comprehensive prosthetic treatment plan developed for an edentulous patient with achondroplasia, underscoring the importance of a personalized, patient-centred approach to effectively restore oral function and aesthetics in the context of this complex skeletal disorder.