Achondroplasia also known as “chondrodystrophia fetalis” is a rare disorder that affects the growth of long bones and cartilages. This disorder is characterized by dwarfism with marked disproportionate short limbs. Considering the complications that may emerge during the treatment of a patient with achondroplasia, it is pertinent that the pediatric dentist should be familiar with the marked features of this syndrome as dental management is duly restricted by practical problems associated with this condition. Patients with achondroplasia not only require specific medical management but also need special attention with definite dental management along with psychological support to help them lead a normal life and cope up with the medical and social challenges of life. We report in this paper about children aged between 3 years and 9 years with achondroplasia with extremely short stature, frontal bossing, macrocephaly, and various dental problems.