To assess management of non-syndromic dens evaginatus affecting permanent maxillary central incisor, we performed a systematic review and also present a case report. We searched PubMed via MEDLINE and the reference lists of included reports. Eligible studies were any type of clinical studies describing the management of non-syndromic dens evaginatus affecting the crown of a permanent maxillary central incisor. We included 31 studies corresponding to 34 relevant case reports. Therapeutic options were complete reduction of the talon cusp in a single appointment (56%), periodic and gradual reduction of the cusp (26%), abstention (13%) or extraction (5%). We report an 8-year-old girl with unusual two-talon cusp, labial and lingual, on a right maxillary double central incisor. A multidisciplinary approach is key to management of permanent maxillary central incisors affected by coronary anomalies.