Macrodactyly and epidermal nevus

A case is presented of a child with the two congenital anomalies of macrodactyly and epidermal nevus. Macrodactyly was noted at birth; nevus unius lateris became apparent at three months. The chance association of these two rare anomalies as individual sporadic occurrences is exceedingly unlikely. The case is presented as a possible syndrome entity, if other case documentation exists.