Scrotoschisis, the congenital extrusion of the testis through the scrotal wall, is very rare. We describe a new case and review the pertinent published data. A male neonate presented with left scrotoschisis that was repaired successfully with a Denis-Browne trans-scrotal orchiopexy, with antibiotic coverage. The testes were intrascrotal and symmetric after 2 years of follow-up. Most cases of scrotoschisis are unilateral and affect normal males. The immediate prognosis is good, but long-term results are not available. The etiology of the disease is unknown, but it might be related to spontaneous healed fetal intestinal perforations with meconium peritonitis.