Albinism is a congenital hereditary anomaly caused by a biochemical lesion in pigment metabolism. The biochemical anomaly consists in the failure of the melanocyte to synthesize normal amounts of tyrosinase.1Albinism is classified as: Complete generalized: complete absence of pigment in eyes, hair, and skin Incomplete generalized: incomplete absence of pigment in eyes, hair, and skin Ocular: incomplete absence of pigment in eyes only Partial2: congenital circumscribed hypopigmented areas in skin and hair, eyes not affected Vitiligo2: variable discrete depigmented areas with hyperpigmented borders which begin at any age; eyes not affected Introduction Figure 1 shows the translucent irises of a dark-haired boy with ocular albinism. Transillumination of the sclera will frequently suggest a diagnosis of ocular albinism or incomplete generalized albinism of a patient who has amblyopia and nystagmus and often a tropia as well as a high refractive error. The incidence of albinism in the United