Testicular cancer is quite rare, making up only 1–2% of male cancers globally. Within this category, Leydig cell tumors (LCTs) were once considered uncommon, accounting for just 1–3% of all testicular tumors removed annually. However, recent studies suggest that LCTs might be more prevalent than previously thought, representing 14–30% of all surgically removed testicular tumors, and up to 45% of non-palpable lesions. Most LCTs are incidentally detect during testicular ultrasounds performed for unrelated indications, such as evaluation of male infertility, testicular hypotrophy, cryptorchidism, or gynecomastia. This suggests that testicular dysgenesis syndrome (TDS) might contribute to the development of these tumors. Due to their hormone-producing nature, Leydig cell tumors may occasionally cause clinical symptoms such as gynecomastia, breast tenderness, early puberty, infertility, low testosterone, or erectile dysfunction. It used to be thought that about 10% of LCTs were malignant. However, advancements in andrological assessment and imaging techniques have led to earlier, and often incidental, detection of LCTs, resulting in a revised estimated prevalence of malignant LCT of approximately 2.5%. Multiparametric imaging has improved the differential diagnosis of intratesticular lesions and, in particular, LCTs often display characteristic patterns on contrast enhanced ultrasound and magnetic resonance imaging. If there is a strong suspicion of a Leydig cell tumor based on clinical and radiological evidence, tumor markers are within normal limits and the lesion is smaller than 1.5 cm, with sufficient residual testicular volume, active surveillance with regular ultrasounds or testis-sparing surgery should be considered. In patients who present initially with hypogonadism, Leydig cell dysfunction may continue even after the removal of the tumor and 40% of men may require testosterone replacement therapy postoperatively.