Malignant transformation in congenital sebaceous naevi in childhood

Sebaceous naevi are uncommon congenital skin lesions with a well-recognised potential for neoplastic change. They should be considered premalignant le sions as malignant degeneration, most commonly basal cell carcinoma and squ amous cell carcinoma, occurs with a lifetime risk of between 5% and 22%. Th is incidence is equal to that of actinic keratosis and exceeds that of oral leukoplakia. Such change, however, is rare before puberty. Basal cell carc inoma may develop in children with naevoid basal cell carcinoma syndrome, x eroderma pigmentosum and rarely de novo but sebaceous naevus is the only so litary lesion in childhood associated with the development of basal cell ca rcinoma. We present two cases of malignant transformation in a congenital s ebaceous naevus occurring in childhood and review the literature and discus s the evidence upon which to base management guidelines.