In November 2014, a review of literature concerning prevalence data of Molar Incisor Hypomineralisation (MIH) and Hypomineralised Second Primary Molars (HSPM) was performed. A search of PubMed online databases was conducted for relevant articles published until November 2014. The reference lists of all retrieved articles were hand-searched. Studies were included after assessing the eligibility of the full-text article. Out of 1078 manuscripts, a total of 157 English written publications were selected based on title and abstract. Of these 157, 60 were included in the study and allocated as 52 MIH and 5 HSPM, and 3 for both MIH and HSPM. These studies utilised the European Academy of Paediatric Dentistry judgment criteria, the modified index of developmental defects of enamel (mDDE) and self-devised criteria, and demonstrated a wide variation in the reported prevalence (MIH 2.9-44 %; HSPM 0-21.8 %). Most values mentioned were representative for specific areas. More studies were performed in cities compared with rural areas. A great variation was found in calibration methods, number of participants, number of examiners and research protocols between the studies. The majority of the prevalence studies also investigated possible aetiological factors. To compare MIH and HSPM prevalence and or aetiological data around the world, standardisation of such studies seems essential. Standardisation of the research protocol should include a clearly described sample of children (minimum number of 300 for prevalence and 1000 for aetiology studies) and use of the same calibration sets and methods whereas aetiological studies need to be prospective in nature. A standardised protocol for future MIH and HSPM prevalence and aetiology studies is recommended.