Background <p>Behçet’s disease is a systemic inflammatory disorder that frequently presents with ocular involvement in the form of non-granulomatous panuveitis and retinal vasculitis. A prepapillary inflammatory vitreous infiltrate represents a rare but potentially characteristic manifestation.</p> Case report <p>We report the case of a 29-year-old female patient presenting with acute, painless visual loss in the right eye. Clinical examination revealed non-granulomatous anterior uveitis with bilateral vitritis and a prepapillary inflammatory vitreous infiltrate in the right eye. Optical coherence tomography (OCT) demonstrated a typical mushroom-shaped prepapillary infiltrate associated with serous macular detachment. Fluorescein angiography revealed optic disc and peripheral leakage. Infectious causes were excluded. In the context of recurrent oral and genital aphthous ulcers, the patient fulfilled the diagnostic criteria for Behçet’s disease.</p> Treatment and outcome <p>Following intravenous high-dose corticosteroid pulse therapy, treatment with adalimumab was initiated. This resulted in significant functional and anatomical improvement, with regression of the prepapillary inflammatory vitreous infiltrate and the serous retinal detachment.</p> Conclusion <p>A prepapillary inflammatory vitreous infiltrate is a rare but important diagnostic sign of ocular Behçet’s disease and may be a key finding, particularly in patients presenting with initial ocular involvement. Early diagnosis and prompt initiation of immunosuppressive therapy are essential for visual prognosis.</p>