We describe three cases of X-linked juvenile retinoschisis confined to the posterior pole of the eye, one of which surgically treated. In the first two cases, in vivo optical coherence tomography (OCT) had revealed a cleavage plane in both nerve fiber and outer retinal layers. In the two similar operated eyes of the third case, postoperative OCT had revealed the disappearance of macular schisis in the eye with good recovery of visual function after surgery. On the contrary, it had revealed a persistent splitting of the retina in the other eye, not clinically visible, which explained the poor recovery of visual function after surgery. In cases with progressive worsening of macular involvement in X-linked juvenile retinoschisis, highly accurate surgery may be considered, and the pre- and postsurgical OCT monitor of these patients is indicated.