Scrotal tumors of nerve origin are extremely rare and occur mostly in the extratesticular tissues of scrotum, such as the spermatic cord and epididymis. A systematic search of the literature in PubMed, Medline, and Google Scholar databases concerning intrascrotal nerve tumors was performed by 2 independent investigators. The systematic search retrieved 45 male adults, with a mean age of included patients at 43.9 ± 18.8 years. The majority of nerve tumors were extra-testicular (86.7%), and only 13.3% originated from the testis. Out of that, 51.1% of neoplasms were histologically proved as schwannomas, 44.4% as neurofibromatosis, and 4.4% as malignant peripheral nerve sheath tumors. The majority of patients presented with atypical symptoms such as scrotal swelling (51.1%), while only 4.4% of patients were asymptomatic. Ultrasonography is the diagnostic modality of choice (97.2%) for the detection of primary lesion, while magnetic resonance imaging and computed tomography comprise supplementary diagnostic tools. Surgical excision of the mass was the preferred type of surgery performed (75.6%), whereas orchiectomy was performed only in 22.2% of patients. Intrascrotal tumors of nerve origin are extremely rare neoplasms that present mainly in middle-aged males. Increased clinical suspicion is required for accurate diagnosis of this rare entity.