A 6-year-old girl presented with right divergent strabismus that had been noticed by her parents 3 weeks before. On examination, she had visual acuity of counting fingers at 3 meters on her right eye and retinal non-regmatogenous bullous detachment with subretinal tumor. Echographic examination disclosed retinal detachment in temporal side from optic disk to equator, a medium reflectivity rounded, homogeneous lesion with rapid growing and positive signs of vascularity. Enucleated eye revealed mixed cell uveal melanoma. This case is a rare presentation of uveal melanoma in which echographic appearance was decisive for diagnosis and treatment.