Dermatofibroma is a common benign cutaneous tumor that usually appears as a slowly growing firm nodule. Polypoid nodular dermatofibroma is a variant type that is rarely encountered. We reported a case of polypoid dermatofibroma with a review of the previously reported cases. Polypoid dermatofibroma tends to arise on the leg, especially below the knee. Its size is often larger than that of common dermatofibroma. It is speculated that both the underlying firm tissue and long-term development may lead the tumor to form a polypoid appearance.