A 13-YEAR-OLD boy, the offspring of first cousins, presented with long-standing, brown-black, hyperpigmented, velvety, hyperkeratotic plaques over both ankles, axillae, neck, and groin (Figure 1). His face was acromegaloid and he had a generalized paucity of subcutaneous fat (Figure 2). His muscles were prominent and his abdomen distended (Figure 3). In addition, he was hirsute and his genitalia were enlarged. A paternal uncle has had similar skin lesions since he was a child.