Congenital enlargement of one or several digits of the hands or feet (macro
dactyly) is a rare disorder, A considerable proportion of the patients with
this condition are referred to dermatology departments. The majority of th
e cases reported in the literature represent hamartomas with combined hyper
trophy of several, predominantly lipomatous, soft tissue components and ove
rgrowth of bone, The differential diagnosis includes Klippel-Trenaunay-Webe
r syndrome, neurofibromatosis, Milroy disease, and Proteus syndrome, We des
cribe eight cases of congenital macrodactyly, discuss the findings, and pro
pose a simple clinicopathologic terminology.