睫状体髓上皮瘤是一种源自神经上皮层的恶性肿瘤，易因其伪装特性而被漏诊和误诊。本文报道了1例3岁9个月的男性患儿，眼部表现似晶状体破裂，经部分肿物切除活检，病理诊断为睫状体恶性髓上皮瘤。

Medulloepithelioma of the ciliary body is a kind of malignant tumor which arises from neuroepithelium. It is easily misdiagnosed or miss diagnosed due to its masquerade feature in the clinical practice. We report a boy with a tumor in his right eye at age of 3 years and 9 months presented first with lens rupture. Diagnosis of malignant medulloepithelioma of ciliary body was made pathologically by biopsy of the partial tumor.