Ectopic scrotum and ectopic testis are both rare conditions. This report describes a 9-year-old crossbreed male dog with both abnormalities, which also has T721C transition in the gene of epidermal growth factor receptor resulted in a Tyr241His substitution in the peptide chain and the second silent A111G transition. High level of total estrogens (101.0 pg/ml), normal testosterone level (2.15 ng/ml) in blood, prostatic hyperplasia (46x44 mm) and hyperplastic epididymis were observed. Moreover, both testicles had signs of connective tissue hyperplasia in epididymis, vasodilation of pampiniform plexus and impairment of spermatogenesis in the left testicle. Both scrotums consisted of the same layers. This constellation of anatomical and genetic anomalies has not been previously described. We postulated that T721C polymorphism in the Egfr gene is one of the genetic factors in polygenic etiology of ectopic scrotum.