Ovotesticular disorder of sex development (DSD) refers to the co-presence of testicular and ovarian tissue in one individual. Here we report a case of Ovotesticular Disorder of Sex Development in a 42 years old male, presented with abdominal mass and undescended testes who had a normal karyotype. Gonadal dysgenesis should always be kept a possibility in patient with undescended testis. Diagnosis relies on clinical findings, hormonal analysis, gonadal histology, chromosome analysis, and genetic testing.