Adenoma of the nonpigmented ciliary epithelium developed in a 36-year-old woman, causing decreased visual acuity and a total cataract in the involved eye. Ultrasound biomicroscopy disclosed associated persistent hyperplasic primary vitreous (PHPV). Sectoral cyclectomy with removal of the mass and intracapsular cataract extraction were performed, and the tumor was submitted for histological examination. Although ciliary adenomas arising in adults are considered to be acquired, the associated PHPV suggests a congenital origin in view of the tumor's possible interference in the normal replacement of the primary vitreous by the secondary vitreous.