Basal cell carcinoma (BCC) is usually seen on the face as a pigmented nodule. We herein report a patient who presented with a polypoid BCC on the nose tip. Clinically, we suspected adnexal tumor; however, the findings of dermoscopy were consistent with that of BCC. Although the tumor was excised at the stalk, it was completely resected. Since the clinical manifestation was characteristic, we reviewed previously reported polypoid BCCs and found that this tumor can occur at any site, including the trunk and inguinal region, which are not preferential sites for ordinary BCC. There have been no reports of polypoid BCC on the nasal tip. The initial diagnoses varied, including adnexal tumors, and dermoscopic examinations proved useful for suspecting polypoid BCC. Histopathologically, the tumor cells in the resected specimens were within the polypoid area. Although BCC is a common tumor, polypoid BCC has distinct clinical features, and we should keep this rare subtype in mind.