A 29-year-old man presented with a large, asymptomatic, brown, hyperpigmented, depressed plaque over his left upper back, which included the scapular area, since childhood. Histopathological analyses of the biopsy specimens was consistent with a rare entity known as neurovascular hamartoma. This uncommon lesion has been reported in two publications, either as a possible marker of the malignant rhabdoid tumor or as a hamartomatous tongue lesion in children. Due to its possible association with the aggressive and often fatal rhabdoid tumor, periodic examination of this lesion may be warranted.