Macular heterotopia secondary to proliferative diabetic retinopathy has been previously reported in clinical cases. To our knowledge, we present the first clinicopathologic case of macular heterotopia in a young patient with proliferative diabetic retinopathy. The significant pathologic findings included dense fibrovascular tissue on the disc to correspond with nasal tractional retinal detachment, a superonasally displaced fovea, and an area of stripped and recoiled internal limiting membrane overlying a retinal fold. In addition, an area of reduplicated retinal pigment epithelium was noted corresponding to the clinically observed hyperpigmented area superior to the heterotopic fovea. Despite the pathologic changes, the patient maintained 20/40 visual acuity with the heterotopic fovea for several years. The findings are discussed in relation to previous clinical studies.