Ovarian cancers arising in children and adolescents are rare events and can be associated with various cancer predisposition syndromes (i.e., genetic conditions that heighten the risk of developing cancers throughout life). Although germ cell tumours are the most frequent subtype of ovarian cancer seen in the pediatric age group, a multitude of other tumours can develop, each one so rare that it may only be seen once in a doctor’s career.

This review article aims to facilitate doctor’s ability to recognize each ovarian cancer type developing in childhood and discusses the features of tumour presentation, patient and family history details that should raise suspicion for specific cancer predisposition syndromes. (By Catherine Goudie, http://jmg.bmj.com/content/early/2017/11/24/jmedgenet-2017-104926 )