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PURPOSE To report the management and outcome of VKH disease in a 4-year-old boy with a review of literature on VKH in preschool children (3 to 5 years). METHOD Retrospective chart and literature review. RESULT A 4-year-old boy presented with visual acuity of light perception in both eyes with panuveitis, secondary cataracts and intraocular pressure (IOP) of ≥40 mmHg in both eyes. He was started on oral corticosteroid, immunosuppressive and underwent glaucoma filtering surgery and lensectomy with vitrectomy after adequate control of intraocular inflammation. Over time his IOP was again uncontrolled even with maximum anti-glaucoma medications, and he underwent Ahmed glaucoma valve (AGV) implantation, and he gained a visual acuity of 20/60 in both eyes with aphakic correction at the last visit. CONCLUSION Paediatric VKH follows an aggressive disease phase and requires a comprehensive multispeciality management approach.