Fusion is the one of the most common dental anomalies in the primary dentition and it is very rare when it appears on the maxillary bilateral incisal areas. The etiology of fusion is unclear. The case was a 4 year-old boy with caries involvement on the bilateral maxillary fused primary incisors. The right maxillary fused tooth had to be treated with endodontic treatment due to caries extending to the pulp. The root canals of fused tooth were thoroughly debrided under the exact differentiation of the root canal morphology. The presence of fused tooth in the primary dentition is associated with a high of degree of anomalies of the permanent dentition. Early diagnosis of the anomalies is, therefore, of considerable importance, followed by careful clinical and radiographic observations and adequate intervention in the appropriate time.