Abstract： Introduction::Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder characterized by developmental malformations and carcinogenic activity. Multi-systemic anomalies may occur in this syndrome, such as odentogenic keratocysts of the mandible and postnatal tumors, especially multiple basal cell carcinoma.Case presentation::A 60 year old man presented with systemic plaques and nodules for more than 30 years. Cutaneous examination revealed that invasive erythema and black papules scattered on the face, trunk and limbs. He underwent extended surgical excision of lesions at multiple sites. No new lesions were found in the treated areas during a 3-year follow-up. Topical 5-aminolevulinic acid-mediated photodynamic therapy (ALA-PDT) was given as a palliative treatment for ulcerated tumors on the right lower eyelid and bleeding of the right temporal lesion. After application of ALA-PDT, the ulcers almost healed and the recurring hemorrhage ceased.Discussion::The management of multiple tumors in patients with nevoid basal cell carcinoma syndrome is extremely difficult, especially in the face. Surgical excision is the standard method, but the procedure can be applied in limited sites and may result in significant disfigurement and the difficulty of wound recovery. PDT can play a significant role in combination therapy for tumors that are extremely difficult to remove completely by surgical excision.Conclusion::We present a rare case manifested as multiple basal cell carcinoma and palmar pits who treated by surgery combined with topical 5-aminolevulinic acid-mediated photodynamic therapy. The combined therapy plays a complementary role in the treatment of nevoid basal cell carcinoma syndrome.