Eccrine angiomatous hamartoma (EAH) is a benign malformation characterized by a proliferation of eccrine glands and capillary vessels in the dermis. Hyperplasia of other dermal constituents, such as fat, nerve fibers, pilar structures and dermal mucin, has been reported. EAH typically presents as a painful lesion on the extremities of children or young adults and may be associated with local hyperhidrosis. We report a case of a 7-year-old boy with a keratotic lesion on the ankle, present since birth. Histologically, there was a nodular proliferation of eccrine glands intimately admixed with numerous small vessels in the dermis. In addition, there was marked epidermal hyperplasia associated with increased numbers of dilated, thin-walled vessels in the superficial and mid-dermis. The vessels were negative for glucose transporter-1 protein (GLUT-1), supporting the impression of hamartoma over that of hemangioma. EAH has been described in association with spindle cell hemangioma and arteriovenous malformation; overlying verrucous epidermal features have been noted in rare cases. However, changes resembling verrucous hemangioma associated with EAH, as seen in this case, have not been emphasized in the literature. The findings are unusual and expand the histological spectrum of this hamartoma.