Purpose: To present a rare case of morning glory disc anomaly in association with an ipsilateral low grade glioma. Observations: A 5 year old male presented with a unilateral morning glory disc anomaly and an ipsilateral sporadic optic pathway glioma with chiasmal involvement. After a strict patching regimen his vision improved from 20/400 to 20/80. Conclusions and importance: This report strengthens the recommendation for brain magnetic resonance imaging in patients with morning glory disc anomaly. Patching of the contralateral eye should be attempted since the role of amblyopia may be significant.