We reported an unusual case of choroidal melano-cytoma who was enucleated with diagnosis of retinoblastoma. A 14-month-old boy has been referred to the clinic with compliant of left eye exodeviation for about 10 months. Ocular examination and imaging work-up revealed retinal detachment with a calcified lesion. The patient underwent enucleation of the affected eye with diagnosis of retinoblastoma. Histopathological findings indicated large, polyhedral shape cells of the tumor with small nuclei and abundant cytoplasm filled with melanin granules, suggesting diagnosis of melanocytoma of the choroids and ciliary body. Many cases of the simulation of the malignant melanoma of the choroids and ciliary body with melanocytoma has been reported previously, but melanocytoma resembling as a calcified lesion and retinal detachment with diagnosis of retinoblastoma is a rare condition which has not been reported anywhere.