Testicular Regression Syndrome (TRS) is defined as the absence or an incomplete development of the testis of varying degrees in 46XY patients with normal external genitalia. The prevalence ranges from 3-20% of cases previously diagnosed as cryptorchidism. We report the case of a 7-year-old boy who underwent surgical exploration with an initial diagnosis of cryptorchidism. Testicular structure was not identified and presumed testicular remnants were sent for histological analysis. The histological sections showed a fibrovascular nodule, structures of the spermatic cord and calcification, supporting the diagnosis of TRS.