We report a rare case of constriction ring syndrome in a 3515 grams full term female newborn who was born with a constriction ring encircling the fingers of her right hand diagnosed as acrosyndactyly. It is a rare congenital malfor- mation and the risk factors are still unknown. The neonate had severe swelling of her third and fifth fingers, and skin breaches. There were intrauterine amputations in her second and forth fingers. The X-ray images showed deformities in both the size and shape of the bones of her fingers. No additional abnormalities or comorbidities were found. The neonate was referred to a better equipped hospital because of impending necrosis from autoamputation. During anamnesis, the mother recalled that she took haloperidol and trihexyphenidyl until 15 weeks’ gestation. There was neither history of fever, trauma, intake of herbal preparations, nor family history of deformity. Etiology and risk factors of congenital constriction ring syndrome are still under discussion. Early intervention is recommended for a successful outcome.