The pit of the disc is a congenital anomaly of the optic nerve disc. The prevalence is 1/11 000 patients. On the affected side, the optic disc is in 85 % of cases larger than the disc of the other healthy eye. The pit of the disc is very often associated with the presence of the cilioretinal artery. Maculopathy in congenital pit of the optic nerve disc was described in the early 30's of the Iast century by Calhoun. The average age of the patients is roughly 30 years of age (20-40 years). The complementary examination method, which may help to clarify anatomical conditions of the macular region, is the optical coherence tomography. The defect of the optic disc of different depth caused by the pit and maculopathy caused by retinoschisis communicating with the temporal rim of the disc are found. This čase report refers to a 29 years old man with disturbing relative centrál scotoma and decreased vision for one month in his right eye, who underwent classical three-ports pars plana vitrectomy with expansive gas tamponade. On the basis of differential diagnosis discretion, the temporally localized pit of the disc accompanied by maculopathy due to retonoschisis was detemined. The surgical treatment by means of three-ports pars plana vitrectomy and peeling of the inner limiting membrane with expansive gas tamponade restored in our patient the physiological macular structure followed by improvement of the best-corrected visual acuity. No complications were noticed during the surgery or after it as well. Among the differential diagnoses, it is necessary to eliminate other possible causes of maculopathy in young patients as well as other congenital anomalies of the optic disc, which may be related to the maculopathy. Maculopathy following the pit of the optic nerve disc represents relatively rare diagnostic entity. According to the literature, the natural course of this disease results in very low finál best-corrected visual acuity, often worse than 5/50 (0,1 or 20/200). The therapeutic possibility for patients with this disease is operative approach by means of pars plana vitrectomy with peeling of the inner limiting membrane and accompanied by expansive gas tamponade as already mentioned in our case report.

        Key words: pit of the optic nerve disc, maculopathy accompanying the pit of the optic nerve disc