Background: Cancer of the penis is an uncommon malignancy in developed countries, but the incidence is as high as 17% of all male cancers in some undeveloped countries. The surgical management of this disease has improved due to better knowledge of risk for metastasis and newer imaging technologies to assess the regional lymph nodes. Methods: We review the literature on incidence, etiology, pathology, clinical presentation, staging, and management of penile cancer. We present our institutional experience with 160 patients who underwent extended ileoinguinal lymph node dissection, as well as with 7 patients who underwent a modified lymph node dissection. Results: Better understanding of pathologic features allow for stratification of patients into low, intermediate, or high risk for lymph node involvement. Lymphatic mapping to this stratification improves selection of patients who might benefit from lymph node dissection after excision of the primary lesion. Our experience with lymph node dissection yielded a high incidence of positive lymph nodes when lymphadenopathy was present. The recent use of a modified lymph node dissection has minimized morbidity. Current chemotherapy agents are ineffective in this disease. Conclusions: Pathologic features of the primary lesion and the incorporation of lymphatic mapping have improved the selection of patients who might benefit from lymph node dissection. The use of a modified lymph node dissection in selected patients has decreased morbidity. Effective chemotherapy agents are needed in the management of advanced penile cancer.