A clinical and histological study was made of a congenital lesion on the knee of a 3-year-old boy showing a peculiar combination of eccrine, pilar and angiomatous structures with the characteristics of organoid nevus. A discussion is made of different questions related to eccrine hemartomas with respect to the clinical picture, the association of other hamartomatous components, the alterations that these induce in other cutaneous structures and their histogenesis.