[Angiokeratoma circumscriptum]

We have presented a 12-year-old boy with angiokeratoma circumscriptum on his left knee. The lesion first appeared at the age of eight as a dark brown nodus with the verrucous surface. It bled easily even after mild trauma this being a principal reason for the hospital admission and surgical excision in toto. Pathohistological examination confirmed the clinical diagnosis. Differential diagnosis, clinical and pathohistological findings, as well as therapy of the disease have been discussed in some detail.