Here we report a case of choroidal malignant melanoma in a 10-month-old child, who has cafÉ-au-lait spots on the abdomen. She had suffered from enlargement of the eyeball since 5 months old due to a tumor-like mass in the vitreous cavity. It was examined with slitlamp biomicroscopy, indirect ophthalmoscopy, orbit computerized tomography and magnetic resonance imaging (MRI). Retinoblastoma or neurofibroma was suspected from the clinical diagnosis, and from the tomographic point of view, it could be choroidal hemangioma, however, the MRI study, unexpectedly suggested the possibility of choroidal malignant melanoma, and it could be confirmed by the results of a histopathologic examination.