Introduction A unique oculocutaneous pigmentation characterized by benign mesodermal melanosis of the paraocular skin and usually the sclera and other ocular tissues was first described and defined as an entity by Ota18 and Ota and Tanino,19 under the name of nevus fuscocoeruleus maxillofacialis. Subsequent authors have called it nevus fuscocoeruleus maxillo-facialis of Ota, or, simply, the nevus of Ota. The latter name is the most often used. The terms oculocutaneous melanosis and oculodermal melanocytosis have also been coined to designate the condition. The former of these, we feel, is too easily confused with melanosis oculi, a different condition, as described by Reese.22,23 The latter, proposed by Fitzpatrick,9 although graphic, is not widely used. Most of the literature pertaining to this subject has been presented from the dermatologic standpoint and the ocular findings have been, with certain exceptions, incompletely reported. It is the purpose of this paper