To describe pathological features and determine the types and frequency of pseudoretinoblastoma simulating retinoblastoma in 70 enucleated eyes.Retrospective histopathologic analysis of consecutively enucleated eyes for presumed intraocular retinoblastoma during the past 11 years in the Second People's Hospital of Yunnan Province in China.9 of the 70 cases were found to be pseudoretinoblastoma on histological analysis. 4 cases were Coat's disease, 2 cases were uveitis and the rest 3 cases were respectively identified as vitreous hemorrhage, toxocariasis and retinal dysplasia. Clinical features and pathological features of these cases were described in detail.Coat's disease accounted for the most common conditions (44.5%) of pseudoretinoblastoma, uveitis (22.2%) accounted for less common conditions, toxocariasis (11.1%), vitreous hemorrhage (11.1%) and retinal dysplasia (11.1%) presented in a sporadic form in our pseudoretinoblastoma series. They have distinctive histopathological features to differentiate from retinoblastoma.