To assess management of non-syndromic double tooth affecting permanent maxillary central incisors, we performed a systematic review and also present 2 case reports. We searched MEDLINE via PubMed and the reference lists of included reports. Eligible studies were any type of clinical studies describing the management of non-syndromic double tooth affecting the crown of a permanent maxillary central incisor. We included 68 studies corresponding to 72 relevant case reports. Therapeutic options in descending order of priority were restorative dentistry (35%), hemisection (33%), abstention (17%) or extraction (15%). Orthodontic management resulted in 57% of cases. We report an 11-year-old boy with bilateral fusion of the two maxillary central incisors and a 9-year-old boy with a double left central incisor and a supernumerary lateral right incisor. A multidisciplinary approach is key to management of permanent maxillary central incisors affected by coronary anomalies.