Macular coloboma is a congenital retinochoroidal defect of the macular region. It is a bilateral, nonprogressive condition that is associated with decreased visual acuity and nystagmus. A case of a 14-year-old boy with an unusual presentation of extensive macular colobomata is described. Location of the lesions differentiated this case from true or typical retinochoroidal colobomata. Differential diagnosis also includes postinflammatory scarring.