Abnormal renal development results in congenital anomalies of the kidney and urinary tract. As many studies suggest that renal malformations are more often found on the left side, a meta-analysis was performed on the distribution of five different unilateral anomalies: multicystic dysplastic kidney, renal agenesis/aplasia, renal ectopia, pelviureteral junction obstruction, and non-obstructive non-refluxing megaureter. Of these anomalies, the left side was affected in 53%, 57%, 56.9%, 63.2%, and 62.5% of patients, respectively, significantly different when compared with an anticipated 50% of left-sided anomalies. An exception to this left-side predominance was found in females with combined genital anomalies and unilateral renal agenesis that commonly present on the right side. The exact mechanisms leading to these lateralizations remain to be determined but may involve vascular development, differential gene expression, or susceptibility to environmental factors such as hypoxia. This remains largely speculative, however, illustrating our limited knowledge of embryogenesis in general and nephrogenesis in particular.