An 11-year-old boy was seen for a routine eye evaluation. Corrected visual acuity, ocular adnexa, and extraocular muscles were normal. Slit-lamp examination of the left eye showed multiple elevated, moderately pigmented, dome-shaped iris lesions (Figure). Gonioscopic examination revealed open angles with several bilateral iridotrabecular attachments. Funduscopic examination of the left eye showed a hyperpigmented lesion of the retinal pigment epithelium. General physical examination revealed multiple flat, pigmented skin lesions larger than 5 mm in diameter on his trunk and freckling in the axillary regions. A first-degree relative had similar iris abnormalities.