Superficial angiomyxomas are rare, benign, dermal and subcutaneous rum
ours. We describe a 12-year-old girl who presented with a nodular swel
ling in the midline of her scalp that had been present since birth. Hi
stological examination revealed an ill-defined myxoid lesion within th
e dermis, comprising spindle cells, blood vessels and occasional multi
-nucleate giant cells. Immunohistochemical staining was negative for S
-100, cytokeratin and smooth muscle actin, but focally positive for CD
34. Our patient is unusual in that the angiomyxoma was present at birt
h, which has not previously been described. The importance of screenin
g patients with cutaneous myxomas for cardiac lesions is discussed.