RESUMO
Brown-McLean syndrome not reported in association with homocystinuria and generally occurs in patients with long-term aphakia. Presented as peripheral corneal edema with central clear cornea tends to spare vision without the need of any surgical intervention. The purpose of reporting such a rare condition as a first case of Brown-McLean syndrome associated with systemic disease of homocystinuria. Brown-McLean Syndrome in a patient with homocystinuria generally occurs in patients with long-term aphakia after different modalities of surgical intervention. A high clinical suspicion and regular follow-up is warranted for patients with systemic diseases who are aphakic or present with a subluxated lens for early intervention and better prognosis if needed.