Internuclear ophthalmoplegia (INO) is a rare disorder of conjugate lateral gaze that has been described in a number of neurologic conditions including multiple sclerosis, stroke and less commonly brain tumors. We describe a series of 3 boys (11, 12, 15 years) diagnosed with primary central nervous system tumors (pilomyxoid variant astrocytoma, anaplastic oligoastrocytoma, gliomatosis cerebri) who developed bilateral INO as a manifestation of progressive disease. Time from diagnosis to development of bilateral INO ranged from 13-36 months. All children died of their disease 1-9 months following diagnosis of bilateral INO and had significant dorsal pontine invasion on magnetic resonance imaging at progression. Only one child had brainstem involvement at diagnosis. Our case series highlights this rare ophthalmologic syndrome of bilateral INO in association with tumor progression and provides a literature review of brain tumor associations with INO.