Eccrine angiomatous hamartoma (EAH) is a benign, uncommon cutaneous lesion, often localized to the distal extremities of children. It usually presents as a solitary, asymptomatic lesion that appears as a nodule or plaque, but sometimes it can occur as multiple lesions. Varying degrees of hyperhidrosis and pain have been reported. The histological feature is characterized by a proliferation of eccrine sweat glands and angiomatous channels. Occasionally, other elements are involved, such as mucin-secreting cells, adipose tissue, bony structures, apocrine glands or hair structures. We describe a case of eccrine angiomatous hamartoma with unusual lipomatous involvement.