AbstractPurpose To study the clinical characteristics, outcome and prognosis of very young patients treated for choroidal melanoma at l’ Institut Curie.Methods Retrospective case‐control series. Patients aged ≤16 years were extracted from the database. Patients were treated by enucleation or irradiation depending on tumor size and site. Histology and genomics of the enucleated globes were reviewed. Metastasis and survival were evaluated using the Kaplan‐Meier method.Results From August 1990 to January 2011, 4,857 patients treated for uveal melanoma were prospectively recorded in the database. Fourteen patients (0.3%), 10 girls, 4 boys, mean age 12 years, (median: 14; [range: 2‐16 years]) at onset were extracted. Mean basal tumor diameter was 14 mm (median: 14.5; [range: 6 to 22 mm]). Mean tumor thickness was 7.3 mm (median: 6.4 [range: 2.4 to 16 mm]). One half of patients were enucleated, while the other half was treated conservatively by proton beam or iodine plaque radiotherapy. Histopathology of enucleated globes showed epithelioid cellularity in all patients. Genomic analysis by CGH‐array was available for 4 patients and showed monosomy 3 for 2 patients and 8q gain for 3 patients. Median follow‐up was 91 months [range: 1 to 243 months]. At the end of follow‐up, 13 patients (93%) were alive, one presented metastatic disease and one had died. The eye was retained in all patients treated conservatively.Conclusion Most children had an excellent outcome in terms of survival. An excellent eye retention rate was observed in conservatively treated patients.