Aim: Aim is to demonstrate that surgery can be the best way to reduce the risk of malignancy in choledochal cysts (CC) and how hard can be the diagnosis and the treatment during pregnancy.

Case Report: We report a case of a pregnant young woman (36 week) with a Todani's score II CC. After caesarean, due to increasing jaundice, she underwent magnetic resonance cholangiopancreatography (MRCP) and Endoscopic retrograde cholangiopancreatography (ERCP) that confirmed the diagnosis. Surgical operation consisted in "cholecystectomy, resection of the choledocal cyst and poli-jejunum anastomosis. drainage of the abscess in the iv hepatic segment".

Discussion: This case report highlights the difficult diagnosis and consequently treatment of a CC, especially during pregnancy. A significant association of biliary duct cyst and hepato-bilio-pancreatic malignancy has been reported with an age-related incidence.

Conclusion: Surgery is considered as the best treatment with a close follow-up because of the risk of recurrent cholangitis and malignant degeneration. This case represents also a challenge because of physiological changes in pregnancy and also because of the risk of fetal mortality and maternal morbidity.

Key Words: Choledochal cyst, Colangiocarcinoma, Pregnancy, Todani's score.

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