Objective: To describe a huge congenital cervical rhabdomyosarcoma. Methods: We were recently confronted with a case of fetal solid neck mass arising in one fetus of a twin pregnancy. Prenatally, the cervical tumor was consistent with teratoma, but it was diagnosed histologically as a rhabdomyosarcoma. Genetic amniocentesis showed a mosaic pattern consisting of 46,XY/46,XY,t(2;8)(q35;q21.2). Results: EXIT procedure was proposed to the parents but declined. The twin with huge cervical tumor died in utero at 35 weeks’ gestation due to hydrops fetalis. Conclusion: Fetal cervical rhabdomyosarcoma is an extremely rare condition that has not been previously reported, but should be considered in the presentation of fetal solid neck mass.