Malignant extrarenal rhabdoid tumor is a rare and highly aggressive tumor of childhood. Intraocular involvement by malignant extrarenal rhabdoid tumor has only been described once as a metastasis. No report exists in published literature describing a primary intraocular malignant extrarenal rhabdoid tumor. The authors report the first such case along with its clinico-radiological features and histopathologic and electron microscopic characteristics.
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