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In patients with progressive familial intrahepatic cholestasis (PFIC), partial external biliary diversion (PEBD), which is associated with a permanent stoma, is recommended as first-line therapy, whereas primary liver transplantation (LTx) is restricted to those with cirrhosis. Our aim was to quantify the health-related quality of life (HRQOL) in patients with PFIC and to evaluate whether there is a difference in their HRQOL depending on the surgical approach.A prospective HRQOL study on a consecutive series of PFIC was conducted using Pediatric Quality of Life Inventory 4.0 child-self and parent-proxy reports. Patients with PFIC after PEBD who still lived with their native livers were compared to those after LTx. Both groups were compared to healthy children.A total of 32 patients (53% girls) patients with a mean age of 17.7 ± 7.3 years were studied. Twenty-two had undergone LTx at a mean age of 7.8 ± 3.8 years and 10 had undergone PEBD at a mean age of 4.1 ± 3.9 years. At the time of HRQOL assessment, the mean age was 18.9 ± 7.5 years in the LTx group and 15.3 ± 6.5 years in the PEBD group. Child-self and parent-proxy reports showed no significant difference in HRQOL between patients with PFIC after LTx and those after PEBD except for marginal difference in physical functioning/health (P = 0.07). Except for a lower score in patient school functioning of patients after LTx (P = 0.01), HRQOL-results showed no difference from healthy children in any group.The HRQOL of patients with PFIC after PEBD was similar to those after LTx. The HRQOL in both groups was also similar to that of healthy children. Thus, our data support the current policy of PEBD as primary surgical treatment for patients with PFIC without cirrhosis.