Ocular paraneoplastic syndromes are rare conditions that can affect any part of the eye at any age. Thus, every ophthalmologist should be familiar with their management, as some of them may reveal severe, life-threatening conditions. These consist overwhelmingly of neuro-ophthalmological manifestations, affecting the optic nerve (paraneoplastic optic neuritis), retina (paraneoplastic retinopathy) or neurological pathways generating eye movements (saccadic intrusion, oculomotor palsy, nystagmus...); occasionally, they involve the anterior segment, orbit or uveal tract. As some of these manifestations appear to be quite common and non-specific, any systemic or especially neurologic comorbidities should increase suspicion. Treatment relies first on oncologic management, and then often more targeted therapy for the associated immune involvement.