Angiokeratoma circumscriptum naviforme (ACN) are sharply circumscribed hyperkeratotic vascular lesions that are often located on the lower extremities. It is called as naevoid (naviforme) since it is seen at birth or after birth and it is the rarest type of angiokeratomas. ACN is not related with other systemic diseases. Lesions of the disease are usually located in the lower extremities and are unilaterally localized. In this article, we present a case of five-year-old boy with ACN. During dermatological examination, sharply circumscribed, about 10x20 cm sized, dark red colored, hyperkeratotic vascular lesions in zosteriform distribution on the right knee lateral were observed. ACN was diagnosed after histopathology and clinical examination. We presented this case since the disease is seen very rare and it had typical clinical manifestations.