Aim: The aim of this case report is to describe the dentofacial manifestations of achondroplasia and highlight concerns associated with dental management of pediatric patients with achondroplasia. Background: Achondroplasia is the most common form of skeletal dysplasia (dwarfism) with clinical manifestations including disproportionate limb shortening and stunted stature. The craniofacial characteristics of achondroplasia are relative macrocephaly, depression of the nasal bridge, and maxillary hypoplasia. Special precautions are necessary during dental management of pediatric patients with achondroplasia due to a large head size, implanted shunts, airway obstruction, and difficulty in head control. Case description: A 6 years and 7 months male, the patient was diagnosed with achondroplasia, currently receiving vitamin D, no known drug allergy, and a mixed dentition stage with multiple caries, mouth breather, and a high risk of further caries based on a caries risk assessment due to poor oral hygiene. As the patient was uncooperative and required extensive dental care, dental rehabilitation was conducted under general anesthesia using oral intubation due to nasal obstruction. Future examinations were planned for every 3 months. Conclusion: The current case demonstrated that the characteristics of achondroplasia might cause respiratory, neurological, skeletal, orthodontic, and psychological difficulties. Pediatric dentists who treat these patients must be able to detect these characteristics and difficulties, as dental treatment is limited by practical issues associated with this condition. Clinical significance: The characteristic features of achondroplasia are attributed to skeletal, respiratory, neurologic, orthodontic, and psychosocial issues. The dentist should be aware of the features of achondroplasia, which can potentially restrict dental management.