A 10-year-old Caucasian boy with recessive dystrophic epidermolysis bullosa was admitted at our institution because of a dental chronic abscess and a severe malnutrition. During physical examination, he presented lesions and scars formation on the face, hands, feet, knees, trunk, elbows, scalp, lips, and inside the mouth as a result of repeated episodes of blistering and subsequent healing. His hands and feet showed with absence of fingers and toes due to the advanced degree of deformity. He also presented microstomia, limited opening of the mouth and extraoral chronic abscess on the left side of submandibular region. Intraorally, due to difficulty in performing adequate oral hygiene, several caries lesions, dental plaque, and dental calculus could be found on his teeth. All dental treatment (including restorative treatment, prophylaxis, calculus removal, and dental extractions) was performed under general anesthesia based on the clinical limitations associated with the patient’s disease and his poorly cooperation. A 10-year-old Caucasian boy with recessive dystrophic epidermolysis bullosa was admitted at our institution because of a dental chronic abscess and a severe malnutrition. During physical examination, he presented lesions and scars formation on the face, hands, feet, knees, trunk, elbows, scalp, lips, and inside the mouth as a result of repeated episodes of blistering and subsequent healing. His hands and feet showed with absence of fingers and toes due to the advanced degree of deformity. He also presented microstomia, limited opening of the mouth and extraoral chronic abscess on the left side of submandibular region. Intraorally, due to difficulty in performing adequate oral hygiene, several caries lesions, dental plaque, and dental calculus could be found on his teeth. All dental treatment (including restorative treatment, prophylaxis, calculus removal, and dental extractions) was performed under general anesthesia based on the clinical limitations associated with the patient’s disease and his poorly cooperation.