A 10-year-old boy was referred to our institution for misaligned teeth, seeking orthodontic treatment. His mother reported no history of systemic diseases or trauma, but she had noticed a swelling on the left side of his face for approximately 3 years without any symptoms. On extraoral physical examination, facial asymmetry with swelling on the left side and mandibular deviation at the opening to the same side was confirmed (Figure 1). We noticed caf? au lait spots on his arms, which led us to others in the thorax region (Figures 2A and 2B), in addition to axillary ephelides (Figures 2C and 2D).