The Proteus syndrome is a recently described congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the musculoskeletal system, primarily by hemihypertrophy, macrodactyly, exostoses and kyphoscoliosis, and the skin and the subcutaneous tissue, primarily by pigmented nevi and subcutaneous tumors. These findings are diagnostic features of Proteus syndrome. We report typical manifestations of Proteus syndrome in a 12-year-old boy with brief review of literature.

Congenital hemihypertrophy is a rare idiopathic condition, first described by Meckel in 1822 and represents enlargement of a part or entire side of the body without associated vascular or neurologic disease. Wagner recorded the first case in the 1839. Hemihypertrophy is to be differentiated from hemiatrophy(which involves unilateral subnormal development, muscle weakness, or neurologic deficit) and the syndromes of hemidystrophy. Approximately 25∼50% of the reported cases of hemihypertrophy have been associated with hamartomas or congenital defects, especially genitourinary anomalies. The early detection of asymptomatic intraabdominal tumor in patients with congenital hemihypertrophy is important

We report 3 cases of congenital hemihypertrophy with discussion of the clinical manifestations and associated anomalies, which were rarely reported in domestic literatures.