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becker nevus syndrome is the association of becker's nevus with breast hypoplasia and other ipsilateral bone or muscle changes. it is considered to be a hormone-dependent disorder caused by a disturbance in the activity of the androgen receptor that appears to be increased in becker's nevus, which may influence the development of associated lesions. we present a relevant case of this syndrome due to the rare systematization of the lesions in addition to the exuberant extracutaneous involvement in this case.