Background: Congenital nevi of the retinal pigment epithelium (RPE) may manifest variable degrees of pigmentation. These nevi, which are almost always asymptomatic, can be either solitary or grouped. Torpedo maculopathy is a recently described congenital RPE nevus.
Methods: A review of congenital nevi of the RPE is presented to include torpedo maculopathy.
Results: Torpedo maculopathy is a solitary congenital RPE nevus; it is oval, variably pigmented, and located in the temporal macula. Diagnosis of this lesion is made on the basis of its characteristic shape and location. The etiology may be related to alterations in the choroidal vasculature in the macular area during the embryologic development of the eye. Because of the benign nature of the nevus, yearly evaluations are recommended.
Conclusions: Classification of congenital nevi of the RPE is still evolving. As more is learned, a better system of organizing these lesions will be developed.