Macular coloboma is a congenital atypical coloboma which appears as well-demarcated lesion on the macula, it can be unilateral or bilateral. We report one such case of a 15-year-old boy who presented to us with horizontal jerk nystagmus. His fundus examination revealed a bilateral large 9–10-disc diameter of well-demarcated excavation at the macula with baring of the underlying sclera and thin rim of pigmentation at the margins. Coloboma was spanning between temporal arcades where vessels followed the course along the edges of coloboma. Large macular colobomas are rare and cause nonprogressive decrease in visual acuity.