We describe a 24-year-old man with primary basal cell carcinoma of the caruncle. Clinically the lesion was a whitish, slightly prominent nodule surrounded by fine vessels. No associated cutaneous lesion and no connection to the surrounding skin was present. The lesion was subsequently completely excised, and histopathological examination revealed a solid-cystic basal cell carcinoma of the caruncle. Primary basal cell carcinoma of the caruncle is an extremely rare but distinct entity. To our knowledge, review of the literature has not demonstrated a previous photographically documented case of primary basal cell carcinoma of the caruncle.