Background: Uveal melanoma is the most common malignant primary intraocular tumor in adults, associated with high mortality. Pediatric uveal melanoma generally has a more favorable course. However, when metastasis occurs, therapeutic options are limited. Design: Clinical presentation, treatment, and outcome of children with uveal melanoma diagnosed in Germany and Austria between 2013 and 2024 were analyzed. Results: Since 2013, 12 children were diagnosed with uveal melanoma in Germany and Austria—9 in the choroid, 3 in the ciliary body and iris. Treatment comprised enucleation (5 patients), external beam radiation with protons (2 patients), ocular brachytherapy (4 patients) and endoresection (1 patient). Two patients developed metastasis. A 17-year-old male with liver metastasis deceased 30 months after diagnosis. A 3-year-old male with metastatic choroidal melanoma presented six months after enucleation with metastasis. Chemotherapy followed by nivolumab and ipilimumab, led to complete response. However, immunotherapy caused insulin-dependent diabetes mellitus. Genetic testing revealed a pathogenic constitutional TP53 variant, confirming Li-Fraumeni syndrome (LFS). At 21 months after diagnosis of metastatic disease, he remains in complete response. Conclusions: This report underscores the rarity and diverse presentation of pediatric choroidal melanoma. Diagnosis of LFS in one patient highlights the importance of genetic testing for tumor predisposition and personalized approaches in managing rare pediatric tumors.