Background: This study evaluates patients with congenital aniridia and cataract who underwent phacoemulsification, capsular tension ring placement, and foldable intraocular lens implantation. Methods: In this prospective case series, 10 patients (17 eyes) underwent cataract surgery via a 3.2 mm clear corneal incision. A continuous circular capsulorhexis with <6 mm diameter was employed. A capsular tension ring and HOYA yellow foldable posterior chamber intraocular lens was implanted. All patients wore color contact lenses postoperatively. Paired t.test was used to compare visual acuity, intraocular pressure, and corneal endothelial changes before and after surgery. Results: A single surgeon performed all surgeries. The best-corrected visual acuity improved from value 1.03 +/- 0.27LogMAR preoperatively to value 0.78 +/- 0.26LogMAR postoperatively (p = 0.000). The photophobic symptoms improved significantly after surgery. The mean corneal endothelial cell density before and after surgery was 3280 +/- 473 cells/mm(2) and 2669 (+/-) 850 cells/mm(2), respectively (p = 0.006). None of the patients developed corneal endothelial decompensation or secondary glaucoma after surgery. Conclusions: Treatment of congenital aniridia and coexistent cataract by phacoemulsification, posterior chamber foldable lens implantation, capsular tension ring placement was safe and effective. Use of colored contact lenses in the postoperative period can reduce photophobic symptoms in this group of patients.