The association of a Mongolian spot of the forehead or cheek with congenital ocular melanosis is apparently a familiar entity to dermatologists, but only two case reports of this type of pigmentation have been found in the ophthalmic literature. Pusey,1 in 1916, recorded the association of scleral and facial pigmentation in a case report of a young Chinese student. Jensen and Haffly,2 in 1948, also reported the occurrence of ocular and dermal melanosis in a 47-year-old Chinese man. In 1930, Ota,3 in Japan, noted a form of pigmented nevus found especially on the face and named it nevus fuscocaerulus ophthalmomaxillaris because of its blue-brown color and its frequent occurrence in the skin area of the zygomatic and maxillary divisions of the fifth cranial nerve. This syndrome of ocular and cutaneous melanosis has subsequently been reported in the dermatological literature as the nevus of Ota. We wish to