A 2-month-old child was referred to our clinic for evaluation and management of a luxated lens. Under general anesthesia, an irregularly shaped iris was observed with the presence of a large iris cyst in the anterior chamber. Sector iridectomy with excision of the cyst was performed. Histopathologic examination confirmed the diagnosis of a stromal cyst of the iris. At the 3-year follow-up, there was no evidence of recurrence of the iris cyst or of a secondary cataract. This case report provides evidence that although a primary cyst of the iris stroma is uncommon, it should be considered in the differential diagnosis for an apparent lens luxation.