We report a case of accessory mammary tissue of type 2a (or polythelia
) entirely localized within a Becker's naevus of mixed type in a 32-ye
ar-old Caucasian woman. Polythelia was congenital in origin while Beck
er's naevus appeared during puberty. Renal ultrasonographic studies, l
aboratory examinations and other instrumental investigations were eith
er normal or negative. Although the association of accessory mammary t
issue with Becker's naevus has previously been reported - also in the
form of mammorenal and acromammorenal syndromes - this is the first ca
se characterized by an anatomic overlap between the two anomalies. It
may be the expression of a common disturbance acting early in embryoge
nesis in the specific developmental fields involved.