A 33 yr old man with a history of pilomatrixoma of the scalp, presente
d 5 mths later with a metastatic undifferentiated carcinoma in a subma
ndibular lymph node. The cutaneous and lymph node tumors showed close
histological similarity and features of malignancy. Investigations ove
r 30 mths excluded a primary neoplasm elsewhere and consequently led t
o the diagnosis of malignant pilomatrixoma. These tumors are rare and
most often located on the scalp. Most of the affected patients are mid
dle age males. Wide excision is advised to avoid recurrences.