Purpose: To report refractive management of bilateral lens subluxation case in Marfan Syndrome patient. Methods: Diagnosis based on a history‐taking, a physical examination, and an ancillary test. The refractive state of the patient was examined by an autorefractometer, streak retinoscopy and best‐corrected visual acuity (BCVA), before and after cycloplegic on each eye. Results: A 7‐years‐old girl diagnosed with Marfan Syndrome by the Department of Paediatric Cardiology complained for blurred vision in both eyes. Initial visual acuity of the patient was 0.027 in her right and left eyes. Anterior segment examination showed phacodenesis in both eyes. After cycloplegic refraction with enlarging pupil diameter and performed streak retinoscopy, BCVA in the right eye was 0.040 with a refractive correction of −22.00 diopters (D) and left eye was 0.021 with −20.00 D. After several follow ups, the refractive state remains highly fluctuates due to severe lens subluxation degree, which is seen through a half pupillary area. Consequently, operative approach using intracapsular lens extraction technique is considered. A month after the surgery, the distance visual acuity in right eye was 0.125 and 0.080 in left eye. With a refractive correction of +5.50 D, visual acuity became 0.500, while for the left eye became 0.125 with +5.00 D. Whereas, binocular distance visual acuity with refractive correction became 0.400. Therefore, spectacle was prescribed to achieve optimal visual acuity. Conclusions: The refractive management steps in lens subluxation associated with Marfan Syndrome case is very challenging depends on patient's condition. The intracapsular lens extraction procedure prior to spectacle prescription should be considered as a management of severe lens subluxation in patient with Marfan syndrome, in order to achieve BCVA.