Retinoblastoma is the most common malignant intraocular tumor in children. An estimated 5000 new cases are diagnosed every year, and of these, very few present as intraocular inflammation. Due to the rarity of the disease itself, which furthermore complicated by the rarity of the uveitis-like presentation, this small subset of cases with atypical manifestations is often misdiagnosed and inappropriately managed. Delay in primary management results in suboptimal life, eye, and vision salvage. Intraocular surgery in a child with viable retinoblastoma can lead to extraocular extension and consequent regional lymph node and systemic metastasis. Therefore, atypical manifestations of retinoblastoma have to be excluded in every child presenting with uveitis and hypopyon.