A 23-year-old patient with a history of 1st degree consanguinity presented with finger-count visual acuity at 1 meter in both eyes. Dilated examination revealed an anteriorly displaced microspherophakia in the right eye and aphakia in the left eye. Fundus examination showed myopic staphyloma with diffuse chorioretinal atrophy, while the rest of the findings were normal. Retinal periphery examination identified a palisade at the 6 o'clock position. The axial lengths were 28 mm in the right eye and 27.5 mm in the left eye.