Marfan syndrome (MFS) is a rare autosomal dominant metabolic disorder, caused by mutations in the gene responsible for coding the fibrillin-1 protein. It is characterized by cardiovascular, musculoskeletal and ocular manifestations. The purpose of this report was to present a rare ophthalmologic complication in a patient with MFS. Female, 51 years old, attended the emergency room complaining of severe pain, low visual acuity and hyperemia in the right eye, started two days before. Biomicroscopy: ectopia lentis (luxated) for anterior chamber; Tonometry: 56 mmHg. Hypotensive drugs were prescribed which reduced intraocular pressure (IOP) to 8 mmHg. Referred for emergency surgery (facectomy without IOL implantation) in order to avoid further damage to the cornea and optic nerve. In the post operative period, presented IOP control and improved VA to 20/50. Multidisciplinary follow-up is necessary in patients with MFS, enabling early diagnosis, which can prevent and minimize any complications.