A case of primary basal cell carcinoma of the caruncle is presented and patients presented in the literature reviewed. Clinical features and outcome of a patient with primary basal cell carcinoma of the caruncle is described. Review of 8 other cases identified through literature search with the keywords of "basal cell carcinoma" and "caruncle" is presented.A 67-year-old male patient presented with a 12 months' history of a lesion over the caruncular region. Incisional biopsy of the lesion revealed primary basal cell carcinoma of nodular type. MRI of the orbit identified extension of the lesion into the medial orbit. The tumor was excised, and reconstructive surgery was performed. The patient declined subsequent radiotherapy. No recurrence was detected during the follow up of 33 months. The current patient and 8 other patients with primary basal cell carcinoma of the caruncle were reviewed.The main therapeutic approach for primary basal cell carcinoma of the caruncle is complete excision with tumor-free surgical margins. Adjuvant radiotherapy or chemotherapy may be administered when deemed necessary. © 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.