A 10-year-old girl with dystrophic epidermolysis bullosa (EB) was referred for a new pigmented lesion in the submandibular area. The lesion, approximately 2 cm in diameter, had irregular borders and a suspicious appearance for malignancy. Dermoscopy revealed a multicomponent pattern with multiple colors, ill-defined network, black blotches, streaks, dots, a blue-whitish veil, and granularity at the periphery. The lesion was evaluated as a possible EB nevus, a newly described entity in EB patients. Despite its atypical appearance, no malignant transformation has been reported. EB nevi are large, asymmetrical, and often irregularly pigmented melanocytic nevi that occur in areas of previous blistering. They resemble recurrent nevi, where melanocytes proliferate in areas of trauma. Clinically, dermoscopically, and histologically, EB nevi may mimic melanoma. However, no malignant transformation has been documented. Two theories explain EB nevi development: repetitive basement membrane disruption leading to melanocyte proliferation, or melanocytes floating in blister cavities and proliferating in the microenvironment of epidermal regeneration. Cytokines and growth factors in blister fluid may promote melanocytic proliferation. EB nevi show similarities to recurrent nevi after trauma or incomplete excision. Dermoscopy and reflectance confocal microscopy are useful in diagnosing pigmented lesions, but their role in differentiating recurrent melanocytic proliferations is limited. EB nevi are benign, and clinicians should avoid overtreatment. Dermoscopy is essential for accurate diagnosis and to prevent unnecessary treatment in these fragile patients.A 10-year-old girl with dystrophic epidermolysis bullosa (EB) was referred for a new pigmented lesion in the submandibular area. The lesion, approximately 2 cm in diameter, had irregular borders and a suspicious appearance for malignancy. Dermoscopy revealed a multicomponent pattern with multiple colors, ill-defined network, black blotches, streaks, dots, a blue-whitish veil, and granularity at the periphery. The lesion was evaluated as a possible EB nevus, a newly described entity in EB patients. Despite its atypical appearance, no malignant transformation has been reported. EB nevi are large, asymmetrical, and often irregularly pigmented melanocytic nevi that occur in areas of previous blistering. They resemble recurrent nevi, where melanocytes proliferate in areas of trauma. Clinically, dermoscopically, and histologically, EB nevi may mimic melanoma. However, no malignant transformation has been documented. Two theories explain EB nevi development: repetitive basement membrane disruption leading to melanocyte proliferation, or melanocytes floating in blister cavities and proliferating in the microenvironment of epidermal regeneration. Cytokines and growth factors in blister fluid may promote melanocytic proliferation. EB nevi show similarities to recurrent nevi after trauma or incomplete excision. Dermoscopy and reflectance confocal microscopy are useful in diagnosing pigmented lesions, but their role in differentiating recurrent melanocytic proliferations is limited. EB nevi are benign, and clinicians should avoid overtreatment. Dermoscopy is essential for accurate diagnosis and to prevent unnecessary treatment in these fragile patients.