Gomez Lopez Hernandez Syndrome (GLHS) is a neurocutaneous syndrome consisting of rhombencephalosynapsis, trigeminal anesthesia and partial alopecia of scalp. We report a case of a five-month-old male child who presented with trigeminal anesthesia, hemi atrophy of the left side, alopecia, head banging episodes and bilateral vascularized corneal opacities with nystagmus. MRI showed rhombencephalosynapsis, left sided microphthalmia and ectasia of internal auditory canal. Examination revealed a Siedel’s negative corneal perforation with pseudo cornea in the left eye. Bilateral tarsorrhaphy was carried out, as the preliminary modality of treatment. This report may shed light on the ocular manifestations of the disease, and aid in early diagnosis and intervention.