BackgroundCraniopagus parasiticus is rare with an incidence of approximately four to six cases in 10,000,000 births. In our case, the head of the parasitic twin protruded from the temporal area of the normal twin’s cranium. The parasitic twin had two deformed lower limbs, of which one was rudimentary, and long bones of the bilateral lower limbs and some pelvic bone. Dissection of the mass of the parasitic twin’s body revealed the intestine but no chest organs or abdominal organs. There was a rudimentary labium but no vaginal opening. In resource-limited countries, maternal age or nutritional factors may play a role in craniopagus parasiticus.Case presentationA 38-year-old multigravida (gravida V para IV) woman of Amhara ethnicity was referred from a rural health center to our hospital due to prolonged second stage of labor at 42+1 weeks. On her arrival at our hospital, an obstetrician decided to do a caesarean section because she was unable to deliver vaginally. A live baby girl weighing 4200 g was delivered. The placenta was single and normal. Her Appearance, Pulse, Grimace, Activity, and Respiration scores were 7 and 9 at 1 and 5 minutes, respectively. She appeared to be grossly normal except for the parasitic co-twin attached to her cranium. After a week of extensive counselling and investigation, a successful separation operation was done. Postoperation, she comfortably suckled on the breast and had no neurological deficit. Two weeks after separation she was discharged in a good healthy condition with an arrangement for postnatal follow up.ConclusionsThe causes of craniopagus parasiticus are still unknown due to a rarity of cases and a limited number of studies on it. There have been only nine to ten cases of craniopagus parasiticus, of which only three survived past birth and were documented in the literature. Genetic scientists and researchers continue to investigate this case because they might find explanations for the birth defect, and provide answers to improve the prognosis and the life chances of twins with craniopagus parasiticus.