Lower midline syndrome is a rare congenital anomaly associated with omphalocele. Undescended testes is a congenital absence of one or both testes in the scrotum. Both of lower midline syndrome and undescended testes incidence have a positive correlation with exposure of cigarette and pesticide. Tobacco agricultural environment is a vulnerable area exposed by pesticides and cigarettes. This is the case report to associated tobacco agricultural environment’s exposure with lower midline syndrome and undescended testes. We describe a case of lower midline syndrome and undescended testes that occurs in the tobacco agricultural environment. In this case we found the exposures of paternal heavy smoker, second-hand smoker, pesticides from tobacco agricultural environment and daily use of bug spray in the house. As conclusion, the exposures from tobacco agricultural environment may take a contribution to lower midline syndrome and undescended testes incidence.