The following report is the first documented case of natal teeth associated with a recently described new entity, Pfeiffer syndrome type 3. The clinical manifestations consistent with the spectrum of this rare disorder are described with an emphasis on the concomitant natal teeth. Pfeiffer syndrome type 3 is one of the craniosynostosis syndromes and has been described in only two patients to date. Both mandibular incisors and maxillary molar natal teeth were found. Natal teeth are teeth, which are present in the oral cavity at birth. They are often associated with developmental abnormalities and recognized syndromes. Their incidence ranges from 1 in 2,000 to 3,500 births. The natal teeth found in this infant included both the mandibular primary incisors and maxillary primary first molars bilaterally. The clinical and histological considerations of natal teeth and their management are discussed. The presence of multiple natal teeth is extremely rare.