Sex cord-stromal tumors (SCST) are rare testicular tumors. However, as 10%-12% of SCST are malignant and 20% are metastatic at initial diagnosis, establishment of protocols for diagnosis and follow-up carries most importance. The standard treatment method is inguinal orchiectomy and testis-sparing surgery is recommended for certain conditions. The studies show that retroperitoneal lymph node dissection is seemingly more of a staging tool and aiding to determine the follow-up protocol. The first 2 years are rather important in the follow-up of the disease. Although follow-up protocol could be more vigorous for patients with malignant clinicopathologic features and metastatic disease, it can be determined individually according to the aggressive properties of the disease and patient status. UROLOGY 73: 1165-1171, 2009. Crown Copyright (C) 2009 Published by Elsevier Inc.