Cryptorchidism (CPT) has been suggested to be common in boys with congenital abdominal wall defects (CAWD). It has been hypothesized that the low intra-abdominal pressure in both omphalocele (OMP) and gastroschisis (GS) and brain malformations in patients with OMP contribute to the high incidence of CPT. To determine the incidence of CPT in boys with OMP and GS and to assess the relationship of CPT to the size of the AWD, prematurity, and concomitant anomalies, the hospital records and autopsy reports of 113 boys with CAWD (OMP n = 75; GS n = 38) were reviewed. Twelve (16.0%) boys with OMP had undescended testes; 5 (41.0%) of those had bilateral and 4 (23.5%) intraabdominal undescended testes. The occurrence of CPT in OMP patients did not correlate with the size of the AWD, birth weight, or gestational age. However, congenital cardiac anomalies and cleft lip and palate were significantly more common (P < 0.05) among those with CPT. Two (5.0%) boys with GS, both of whom were born prematurely, had unilateral CPT. In patients with OMP, the incidence of CPT was significantly higher than that of healthy children. There was a correlation between CPT and congenital midline defects such as cardiac anomalies and cleft lip and palate. In patients with GS, the incidence of CPT only slightly exceeded that of normal children and may have been related to prematurity.