Vasoproliferative retinal tumors are benign tumors of unknown origin that generally affect healthy persons between their fourth and sixth decades and may lead to severe vision loss as a result of intraretinal hemorrhages and exudates. They must be differentiated from retinal capillary hemangiomas of von Hippel-Lindau disease, retinal astrocytoma, Coats disease, peripheral exudative hemorrhagic chorioretinopathy, choroidal melanomas, and retinal/choroidal metastasis. We describe a case of unilateral vasoproliferative retinal tumor associated with retinitis pigmentosa in a patient with Usher syndrome type 1.