PURPOSE: Crossed Testicular Ectopia (CTE) is a rare congenital anomaly where both testes descend on one side of the body. Although previously believed to be exceedingly uncommon, the number of published cases has grown, suggesting it may be more prevalent than initially believed. CTE is associated with various abnormalities, of which the most cited anomaly is persistent mullerian duct syndrome (PMDS) which has its own implications regarding infertility. This systematic review aims to clarify the impact of CTE on fertility, histopathology, associated congenital abnormalities, and potential long-term outcomes. METHODS: A systematic review of the literature was performed to identify relevant studies on CTE. Inclusion criteria covered case reports, case series, and meta-analyses with individual case data published in English. Two reviewers independently extracted data, including demographic details, diagnostic methods, histological findings, and fertility status. Data analysis was performed using JMP software. RESULTS: We identified 417 cases of CTE, a significant increase from previous review. CTE was diagnosed preoperatively in only 42.6% of cases, with ultrasound and MRI achieving the highest diagnostic success rates. Histological abnormalities were common, observed in 66% of cases, including testicular dysgenesis, Leydig cell hyperplasia, and malignancy. Infertility was reported in 79.2% of patients, notably high even among those with unilateral undescended testes. Fusion anomalies involving the spermatic cord, vas deferens, or testes were documented in 9.5% of cases. PMDS was the most common associated anomaly, identified in 33.3% of cases, and appeared to reduce the likelihood of fusion anomalies. CONCLUSION: This review highlights CTE as a complex and potentially underdiagnosed condition with significant implications for fertility and cancer risk. Early diagnosis and intervention are essential to improving long-term outcomes, while future research should investigate the genetic factors underlying CTE and optimize diagnostic protocols.