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Background Gonadoblastoma (GB) is a rare tumour of the gonads presenting in childhood or adolescence. It is a lesion composed of a mixture of germ cells at different stages of maturation, with low malignant potential. It is associated with disorders of sex development, most commonly Turner mosaic syndrome with Y chromosome material (TMSY), and 46XY gonadal dysgenesis (GD). Little is known about the natural history and incidence, however prophylactic gonadectomy is recommended. Objectives To determine the incidence and clinical features of GB presenting in childhood in the Irish Republic (RoI) from 1999–2018 inclusive. Methods A retrospective review of children and adolescents with a diagnosis of GB was undertaken using the records of the National Cancer Registry Ireland (NCRI) and the Departments of Endocrinology, Pathology and Surgery at the main children’s hospitals. Results Fifteen cases of gonadoblastoma were identified, all except one phenotypically female. Fourteen patients had prophylactic gonadectomy and one presented with an ovarian mass and raised tumour markers. Eight had TMSY (age at gonadectomy 2 weeks – 14 years). Seven were phenotypically female and one was male. Seven cases of 46 XY GD (all female phenotype) were diagnosed with gonadoblastoma with an age range of 4 months – 15 years at time of surgery. Four of these were unilateral. In the remaining three cases, one patient had bilateral gonadoblastoma, one had unilateral dysgerminoma and contralateral gonadoblastoma and the third had bilateral dysgerminoma with features of gonadoblastoma. Conclusions This is the first reported population incidence rate of GB in children with a 20 year incidence of gonadoblastoma in the Republic of Ireland of 1/100,000 live births. The data supports the recommendation for elective gonadectomy in high risk conditions. Due to the wide age range in presentations, however, the timing of gonadectomy should be individualised, based on underlying diagnosis and following multidisciplinary team discussion. The true rate of malignant transformation in early onset GB remains to be studied.