Macrodactylism or macrodactyly is referred to as congenital deformity of fingers or toes, and the corresponding incidence rate is relatively low. In this article, we describe a young male with macrodactylism of the second toe of his right foot. He was suffering from persistent pain, overgrowth, and hyperhidrosis ofthe involved toe. Radiographic examination of the right foot showed a small translucent area surrounded by hyperplasia and sclerotic bone in the second distal phalanx, in addition to increased soft tissue density and volume. Surgical resection of the bone lesion and reduction of the soft tissue bulk were performed. Pathological findings showed osteoblast hyperplasia, which was diagnosed as osteoid osteoma, and noabnormal findings were seen in the skin. Symptoms of pain and hyperhidrosis disappeared postsurgery and did not recur over the subsequent 2-year follow-up. As far as we know, this was a rare case of osteoid osteoma occurring in the toe that resulted in macrodactylism, which was also associated with localized hyperhidrosis.