Tufted angioma is an uncommon vascular benign tumor of infancy or early childhood with slow and indolent growth. We report the case of a four-year-old boy with an asymptomatic annular lesion over the nape of the neck resembling granuloma annulare. Punch biopsy revealed multiple scattered vascular lobules or tufts. Aggregates of closely packed, dilated to compressed vascular spaces with plump endothelial cell lining were seen in the dermis, confirming the diagnosis of tufted angioma. Immunohistochemical positivity was present with CD31 and CD34 staining of endothelial cells. It is a slow-growing tumor; Kasabach–Merritt syndrome (KMS) and consumptive coagulopathy are rare complications. Blood coagulation profiles were within normal limits. In our case, the lack of reliable distinguishing characteristics of the vascular tumor and atypical annular presentation was a source of diagnostic confusion. Tenderness, typically present in tufted angiomas, was absent in our patient. This unusual case illustrates that clinicians should recognize an annular configuration as an atypical manifestation of tufted angioma.