In orthodontic practice, orthodontist frequently face the patients with congenitally missing maxillary lateral incisors and mandibular second premolar. There are some treatment options for patients with congenitally missing second premolar such as keeping the deciduous second molar, extracting the molars and allowing space to close spontaneously, prosthetic replacement, autotransplantation and orthodontic space closure. Instead of treatment option for congenitally maxillary lateral incisor are orthodontic space opening for future restoration or orthodontic space closure using canines to replace missing maxillary lateral incisors. There are some factors must be consider before making a decision, such as the facial profile, dentoalveolar protrusion, the tooth size-arch length discrepancy, inclination and position of canine, dental esthetic of canines, occlusion, the dental relationship, patient’s age, the stage of development of the adjacent teeth and the condition of the deciduous predecessors with regard to root resorption and infracclusion. In this paper it will be discussed about the indications, advantages and disadvantages of both treatment modalities to help clinicians to cope with the malocclusion cases with congenital missing teeth. In this regard it needed teamwork among the orthodontist, prosthodontist, dental surgeon and restorative dentist to analyzing some factors related to individual patients and establishing overall treatment plans.