Tufted angioma (TA) is an uncommon benign vascular tumor that manifests as erythematous macules, plaques, and nodules and progresses slowly. It is histopathologically characterized by tufts of endothelial cells in a cannonball pattern in the dermis. It primarily affects children and adolescents. A 12-year-old child presented to us with a single, painful, sizeable annular plaque on the right side of his neck that had been there since he was 5 years old. Lupus vulgaris, borderline tuberculoid Hansen, annular elastolytic giant cell granuloma, acquired hemangioma, and benign vascular tumors were all evaluated as differential diagnoses. Ultrasound of the neck revealed an ill-defined hypoechoic region on the right side of the neck with no extension into the deeper plane. TA was diagnosed based on histopathology. We report this case because of its uncommon clinical form of TA, which presented as a single large erythematous annular plaque resembling a granulomatous lesion, which was later confirmed by histological features. © 2025 Elsevier B.V., All rights reserved.