A rare congenital abnormality of the optic disc, known as the optic disc pit (ODP), can cause progressive vision loss when it is associated with maculopathy. Only 15% of ODP cases are bilateral, with a reported incidence of 1 in 11,000 cases and with no gender differentiation. A 56-year-old woman presented in the outpatient department with a history of gradual painless diminution of vision in the right eye for one year. On ocular examination of the right eye, the patient has a vision of counting finger 1 m not improving with pinhole, and anterior segment evaluation is within normal limits. On fundus examination, we could appreciate a grayish, white small hypopigmented depression present in the inferotemporal part of the optic disc suggestive of ODP and edema present in the inferior half of the macula along with cystoid macular edema. The fundus photo of the right eye was suggestive of inferotemporal ODP in the right eye and normal fundus in the left eye. Optical coherence tomography (OCT) of the right eye showed inferotemporal ODP connecting with the subarachnoid space of the optic nerve. The macula showed diffuse edema extending from disc to macula, neurosensory detachment, macular schisis, and cystoid macular edema. We report a case of unilateral ODP maculopathy (ODP-M), which is a congenital anomaly of the optic nerve head (ONH) located at the inferotemporal part of the optic disc with multilayered separation involving all retinal layers and connecting with the subarachnoid space, as seen on OCT.