The occurrence of intermediate uveitis in monozygotic twins has rarely been reported in literature. We report the occurrence of this disease condition in identical twins. Twin sisters presented with typical features of intermediate uveitis, which appeared three years earlier in one of the sisters. They were treated with posterior subtenon injection of triamcinolone acetonide and recovered well. Systemic evaluation and laboratory investigations did not reveal any etiology. Cytogenetic high-resolution analysis did not reveal any chromosomal abnormalities. Pedigree analysis revealed no specific transmission pattern. No other family members in three generations had similar ocular disease. Our report indicates that a detailed family history should be taken in intermediate uveitis and examination of other siblings of patients with intermediate uveitis should be performed.