We describe the clinical presentation, high-frequency ultrasound biomicroscopic characteristics, and pathologic findings associated with a conjunctival inclusion cyst within the iris. The patient had undergone an uncomplicated extracapsular cataract extraction with posterior chamber intraocular lens insertion 9 months prior to presenting with a progressively enlarging iris mass. A clinical examination revealed a solid-appearing white tumor within the midiris stroma, accompanied by limbal-conjunctival hyperemia and anterior chamber inflammation. Ultrasound biomicroscopy revealed an egg-shaped solid iris stromal tumor that displaced the pigment epithelium. The mass was composed of three concentric layers of different echogenicity: a moderately reflective mantle, a less reflective middle zone, and a hyperreflective core. Within 3 days of initiation of topical corticosteroid therapy (prednisolone acetate, 0.5 mg per drop four times daily), the tumor enlarged and induced a plasmoid aqueous and a hypopyon. Histopathologic study revealed a conjunctival inclusion cyst with evidence of acute and chronic inflammation. We have found that the diagnosis of epithelial inclusion cyst within the iris can be aided by an ultrasound evaluation. This case also suggests that it may be preferable to excise these tumors prior to topical corticosteroid treatment.