A 69-year-old man was referred to our hospital with complaints of central vision loss and metamorphopsia in the left eye for three weeks. Several years earlier, the patient underwent vitrectomy and lens reconstruction surgery in the right eye for retinal detachment. The assessment at admission to our department revealed a best-corrected visual acuity of 1.0, with a refraction of − 1.0 diopter sphere with − 1.0 diopter cylinder at 90° in the right eye (OD) and 0.2 with a refraction of − 2.5 diopter cylinder at 80° in the left eye (OS). The axial length was 25.90 mm OD and 25.00 mm OS. The intraocular pressure was 18 mmHg OD and 20 mmHg OS. Slit-lamp examination revealed no abnormality, except for the presence of an intraocular lens in the right eye and mild cataract in the left eye. Dilated fundus examination revealed macular retinoschisis and a suspected macular hole with posterior vitreous detachment in the left eye. The optic nerve head in both eyes exhibited moderate rim thinning with a nerve fiber layer defect at the inferotemporal margin. Retinal breaks with chorioretinal scars were observed on the peripheral fundus of the right eye. Spectral-domain optical coherence tomography (SD- OCT) showed an outer lamellar macular hole with foveal retinal detachment. Retinoschisis was observed at the outer plexiform layer around the fovea and the inner retinal layer between the optic disc and fovea. OCT line scans via the optic disc in both eyes revealed deep cupping of the optic nerve head and posterior displacement of the peripapillary sclera relative to the retinal pigment epithelium. PICCs were observed inside the thickened peripapillary choroid and a honeycomb-like structure was observed in the prelaminar region of the left eye. At the initial evaluation, neither optic disc pits nor communication among the vitreous cavity, PICC, and intraretinal space were observed in both eyes. Based on these observations, the macular lesion was considered to have been caused by an anomaly of the optic nerve head. Our service indicated pars plana vitrectomy with internal limiting membrane peeling and sulfur hexafluoride gas tamponade to address the pathology. During surgery, complete posterior vitreous detachment around the optic disc was observed, consistent with the slit-lamp examination findings at presentation. The fovea-sparing internal limiting membrane peeling (FSIP) technique and subsequent fluid-gas exchange using a 12% sulfur hexafluoride gas tamponade were administered. The patient remained in the prone position for 2 days postoperatively. Two weeks postoperatively, the macular retinoschisis had improved, but the foveal detachment remained. In the third postoperative week, the patient underwent another vitrectomy due to complications, namely, rhegmatogenous retinal detachment with macular detachment. Tamponade using 20% sulfur hexafluoride gas was performed. Postoperatively, no full-thickness macular hole formation was observed and the macular retinoschisis resolved. Corrected visual acuity in the left eye had improved to 0.6. During the three years of follow-up, no recurrent macular lesions were observed. OCT scans of the optic disc of the left eye three years postoperatively demonstrated changes in the curvature of peripapillary retinal pigment epithelium and sclera compared to the baseline OCTs and a communication between the temporal PICC and vitreous cavity, which had not been observed during the initial examination. The patient provided written informed consent for the publication of this case, including images.