In the present literature review, modern view on etiology, pathogenesis, clinical course, diagnosis, treatment and prognosis of scrotal and penile epidermoid cysts is described. Such cysts are rare, refer to benign cystic tumors with slow growth and low malignant potential. Epidermoid cysts develop in different races representatives, mostly in the age range of 30-40 years. Causes of epidermoid cysts remain unclear. Their relationship with different chromosomal aberrations, vulgar acne, post-pubertal age hyperandrogenism, human papillomavirus infection, excessive sun exposure, external genital organs injury are discussed. Epidermoid cysts histogenesis is not completely understood. Preference is given to disembriogenetic theory of the cysts origin. Epidermoid cysts have a characteristic clinical and ultrasound picture, and in the absence of complications have latent and favorable course. Epidermoid cysts diagnosis usually is not difficult and based on cysts characteristic visual picture. In controversial cases, epidermoid cysts must be differentiated from the inguinal-scrotal hernia, lipoma, hematoma, primary malignant or metastatic tumors of the genital organs. In these cases, high resolution ultrasound examination and high-field magnetic resonance imaging are used. Epidermoid cysts complications occur rare. Among them, cases of cysts traumatic rupture with the scrotal hematoma and infection development are the most frequent. Isolated cases of epidermoid cysts blastomatous transformation in various types of skin cancer, T-cell lymphoma, carcinoid tumor, melanoma in situ are described. As the treatment of epidermoid cysts active follow-up in uncomplicated cysts, medication and surgical treatment are offered. Medication treatment is used for inflamed cysts. Surgical treatment is absolutely indicated for abscess formation and suspected malignancy in epidermoid cyst. The prognosis for epidermoid cysts surgical treatment is generally favorable. Cases of epidermoid cyst recurrence in case of their total excision were not observed.