48,XXYY syndrome was initially considered a variant of Klinefelter’s syndrome R1627CIHJAGGF. Nowadays, it is accepted as a distinct clinical and genetic entity R1627CIHEHDJE, R1627CIHBIGGH. Individuals with this syndrome are more aggressive, more intellectually handicapped, and taller than people with Klinefelter’s syndrome R1627CIHBAHHB. Children and adolescents with 48,XXYY syndrome often come to psychiatrists for behavioral problems, sometimes even before the chromosomal diagnosis is made. The incidences among newborn and institutionalized mentally retarded patients are 1/50,000 and 0.08%–0.33%, respectively R1627CIHFHHGI, R1627CIHCJIFG. Behavioral and intellectual disturbances in these patients are likely related to sex-chromosomal abnormalities R1627CIHBIGGH. Clinical signs of 48,XXYY are generally nonspecific. Some of the common features include tall stature, gynecomastia, truncal obesity, skin ulcers, and a craniofacial dysmorphism described as a "pugilistic" facial appearance R1627CIHBHHII.