This is a case of ocular malformations with presumable intraocular calcification based on computed tomography (CT) imaging, magnetic resonance imaging (MRI) and ocular ultrasound (US) findings. The authors presented the clinical, imaging and pathological findings of this case. Intraocular calcification is the most important finding in retinoblastoma, which requires aggressive management. It is important to distinguish it from other intraocular lesions, especially intraocular calcified hematoma.

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