We compared psychophysical and electroretinographic test results of four patients with X-linked juvenile retinoschisis who had clinically apparent lesions isolated to the foveal area. The b-wave of the flash electroretinogram was selectively reduced, while the a-wave was within the normal range. Oscillatory potentials generated by either the rod or cone systems were markedly reduced. Absolute thresholds outside the fovea were either normal or only moderately elevated, indicating that the sensory neural pathways were, by and large, operating with limited dysfunction. Therefore, the oscillatory potentials do not directly reflect, to any appreciable extent, the function of bipolar or ganglion cells. Finally, a sequence of pathologic events is proposed for X-linked juvenile retinoschisis that initiates with Müller cell dysfunction.