Aim : To report the refractive and visual outcome after clear lens extraction with posterior chamber IOL implantation for anterior lenticonus in a patient with Alport syndrome. Method: A 16year old male patient who was referred for amblyopia therapy on account of isoametropic amblyopia was found to have myopic astigmatism of -4.50 cyl and -3.50cyl, with BCVA of 6/60 and 6/36 respectively in the right and left. The triad of anterior lenticonus in both eyes, deafness and ultrasound and biochemical evidence of early kidney disease led to a diagnosis of Alport syndrome. Result: Subsequent clear lens extraction with posterior chamber IOL implantation in both eyes restored vision to 6/6 with no significant refractive error Conclusion: Timely surgical intervention with clear lens extraction and IOL implantation is effective in restoring vision to normal in patients with poor spectacle-corrected vision in Alport Syndrome.