The majority of arachnoid cysts are congenital intracranial lesions that develop in the early embryonic stages as a result of a slight irregularity in the cerebrospinal fluid's (CSF) passage through the embryonic mesenchyme. Most of the time, these cysts are asymptomatic all throughout life. Diplopia caused by an arachnoid cyst is extremely rare. We present a rare event of isolated fourth nerve palsy in a 56-year-old woman brought on by an intracranial arachnoid cyst. Her only presenting symptom was vertical diplopia for one week. She denied any history of trauma. Ocular motility revealed limitation of abduction in her right eye. We proceeded with neuroimaging and the magnetic resonance imaging (MRI) confirmed the presence of a well-circumscribed left retro-cerebellar lesion which follows the CSF signal intensity in all sequences causing compression onto the posterior aspect of the left cerebellum, keeping with the diagnosis of an arachnoid cyst. This uncommon pathology tends to be difficult to diagnose and treat.