Neonatal cholestasis as the presenting feature of cystic fibrosis

Neonatal cholestasis as the presenting feature of cystic fibrosis is rare. We reported a 2 and half months infant who presented neonatal cholestasis since 27 days of life. The punch biopsy of the liver showed signs of cholestasis. Serological tests revealed CMV infection; sweat test showed elevated chloride and confirm the diagnosis of cystic fibrosis. This patient developed a pulmonary pseudomonas infection. The outcome was fatal. Several etiologies may be involved in neonatal cholestasis and the presence of one does not preclude the other