OBJECTIVES: Familial Chylomicronemia Syndrome (FCS) is a rare hereditary form of hyperchylomicronemia, a lipoprotein dysfunction leading to high levels of plasma triglycerides and resulting in various complications, including acute pancreatitis and abdominal pain. It affects about 1-2/1,000,000 individuals worldwide. The purpose of this study was to estimate the health care resource utilization, more specifically hospitalizations associated with FCS patients in a real-life setting. METHODS: A retrospective study was conducted using data from patients who were diagnosed with FCS at the Saguenay-Lac-St-Jean hospitals and clinics between January 1958 and April 2015. Patients were included in the database regardless of their age or the time since diagnosis. Data collection was performed from December 2014 to June 2015. Hospitalizations were confirmed by an exhaustive medical chart review. We focused on characteristics associated with FCS pancreatitis-related hospitalizations (PRH) and abdominal pain-related hospitalizations (APRH). RESULTS: The study included 58 FCS patients (mean age of 43.9 years (SD=15.1), 57.0% men). Thirty-one (53.0%) patients suffered from at least one episode of pancreatitis or from other abdominal pain, among which, 30 and 24 of these patients required at least 1 hospitalization because of PRH or APRH respectively. PRH were more frequent (15.7 hospitalizations/patient) than APRH (4.5 hospitalizations/patient). Furthermore, the PRH rate was higher in women than in men (19.9 hospitalizations/women vs. 12.0 hospitalizations/men). Moreover, pancreatitis and abdominal pain were associated with an average of length of hospital stay of 8.1 (SD=8.5) and 6.5 (SD=7.4) days respectively, and was higher in women than men for PRH (9.1 vs. 6.5 days) and for APRH (9.5 vs. 4.6 days). Based on the current average hospital cost per day in Quebec, Canada, estimated costs is CDN$7,970 per PRH and CDN$6,396 per APRH. CONCLUSIONS: FCS is associated with increased hospitalization resource utilization and cost. Pancreatitis and abdominal pain represent cost-generating complications.