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.