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Fraser syndrome is an autosomal recessive inherited disorder with multiple anomalies that have always been seen with cryptophthalmos. In this study, a 14-year girl with cryptophthalmos, syndactyly of the upper and lower limbs finger, cardiac and urogenital abnormalities referred to ophthalmology department of Kermanshah’s Imam Khomeini Hospital in 2011. There was no visual organized globe in ocular ultrasound examination. Separation of the lens of the anterior and posterior segments was not visible. On both sides of a large cystic space was seen anterolateral globe. Although we did the ultrasound, there was no visual potential, but the patient at the time of the photo flash light response suggests that there is some degree of health of the visual pathway, but these symptoms alone cannot be the reason for the surgery. Therefore, we recommend preoperative ultrasonography and if the confirmation of visual potential and organized globe, lid opening can be done to prevent suffering and unnecessary further surgery.

Keywords: Fraser Syndrome, cryptophthalmos, inherited disorder

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