We present a case report of a patient with basal cell nevus syndrome (BCNS) who developed multiple polypoid basal cell carcinomas (PBCC) in the perineum. PBCC is a rare variant of nodular BCCs. Clinically, PBCCs mimic acrochordons. We recommend that the perineum, perianal, and genital areas should be included in the routine exam of patients with BCNS.
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