OBJECTIVE: To determine the safety and efficacy of surgical versus medical management in the treatment of ophthalmic complications of homocystinuria, and also to document ocular complications of homocystinuria other than lens dislocation. DESIGN: Retrospective case series. PARTICIPANTS: Forty-five patients with ophthalmic complications of homocystinuria participated. INTERVENTION: Eighty-four surgical procedures were performed on 40 patients. There were 82 procedures done with the patients under general anesthesia and 2 with the patients under local anesthesia. Medical therapy was attempted initially in all patients with lens dislocation and was the sole therapy used for five patients. MAIN OUTCOME MEASURES: Complications resulting from medical or surgical treatment and final visual acuity were studied. RESULTS: All patients had a history of lens subluxation or dislocation. Fourteen (31%) were receiving dietary treatment at the time of presentation and 29 (64%) were mentally retarded. Eighty-two procedures were performed with the patients under general anesthesia with 2 surgical complications and 1 postsurgical complication. Lens dislocation into the anterior chamber was the most frequent indication for surgery (50%) followed by pupillary block glaucoma (12%). Prophylactic peripheral iridectomy was not successful in preventing lens dislocation into the anterior chamber in five patients. Anesthetic precautions such as stockings to prevent deep venous thrombosis, preoperative hydration, or aspirin were given in 85% of cases. Other common ophthalmic complications found include optic atrophy (23%), iris atrophy (21%), anterior staphylomas (13%), lenticular opacities (9%), and corneal opacities (9%). CONCLUSION: Laser iridectomy was unsuccessful in preventing lens dislocation into the anterior chamber. With appropriate anesthetic precautions and modern microsurgical techniques, the risks associated with the surgical management of ocular complications of homocystinuria are reduced. Surgical treatment should be considered, especially for cases of repeated lens dislocation into the anterior chamber or pupillary block glaucoma. If a conservative, nonsurgical approach is undertaken, these patients must be observed carefully for repeat episodes of lens dislocation.