Although there have been continuous improvements in child oral health over recent decades, first permanent molars (FPMs) remain susceptible to early caries and can often be affected by hypomineralisation. We highlight current thinking in caries management and the restoration of hypomineralised FPMs, while also discussing enforced loss of these teeth within the context of interceptive extractions or extractions as part of orthodontic treatment. Compromised FPMs can negatively impact on quality of life for a child and present significant management challenges for the dental team. Although a high-quality evidence base is lacking for the different treatment options, early diagnosis and multidisciplinary treatment planning are key to achieving the best outcomes.