Background: A case of a 13-month-old male infant with progressive increase in head circumference, neurodevelopmental delay, and episodic generalized seizures is reported. He had a history of increase in intracranial pressure because of an arachnoid cyst at the age of 2 months which was managed by cystoperitoneal shunting, but the symptoms were still progressive.
Discussion: Magnetic resonance imaging revealed an intraventricular cystic tumor with intensely enhancing mural nodules. He underwent surgery and the cyst with the nodules was totally excised. Histopathological findings confirmed the diagnosis of cystic choroid plexus papilloma.
Conclusion: We recommend precise evaluation of intracranial cystic lesions to prevent wrong diagnosis and operation. Total resection of all enhancing nodules in cystic choroid plexus papilloma is proposed to warrant total cure of tumor.
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