CACNA1A encodes the alpha-1A subunit of P/Q-type voltage-gated calcium channels (Cav2.1) and a transcription factor, critical for neurotransmitter release and Purkinje cell development, particularly in the cerebellum and brainstem. These regions play a vital role in coordinating motor control, including ocular motor function. Mutations in the CACNA1A gene are associated with a spectrum of neurological disorders collectively known as “CACNA1A channelopathies,” including episodic ataxia type 2 (EA-2), familial hemiplegic migraine type 1 (FHM1), and spinocerebellar ataxia type 6 (SCA6). Eye movement abnormalities are common in these disorders, reflecting dysfunction in the cerebellum and its connections to the ocular motor nuclei. Ocular motor control is a complex process requiring precise coordination of multiple pathways to generate saccades, smooth pursuit, gaze-holding mechanisms, and the vestibulo-ocular reflex (VOR). Disruption of these processes leads to distinct patterns of eye movement abnormalities, which can be valuable clinical markers for diagnosing CACNA1A-associated disorders. Common features include nystagmus (e.g., gaze-evoked, rebound, or downbeat nystagmus), saccadic dysmetria, impaired smooth pursuit, and deficits in VOR suppression. This review explores the spectrum of eye movement abnormalities observed in disorders caused by CACNA1A mutations. By understanding these abnormalities, clinicians can improve diagnostic accuracy, provide targeted management, and gain insights into the pathophysiology of CACNA1A channelopathies. Additionally, we aim to highlight how these ocular signs can serve as sensitive markers of cerebellar and brainstem dysfunction, offering clues to the broader neurophysiological consequences of these mutations.