A 15-year-old male born of non-consanguineous parents is referred for assessment of elevated liver enzymes, factors V and VII below lower limit of normal, and multiple liver nodules visible in all liver segments on Doppler US, compatible with FNH-like lesions or haemangiomas. His family history is remarkable for early deaths of unknown cause on the paternal side. His past medical history reveals a developmental delay, left periventricular infarction at birth, multiple episodes of right shoulder dislocation, and a clinical suspicion of Ehler-Danlos with negative genetic workup. His mother reports a bodily fish odour. The physical exam is remarkable for tall stature (>P90), lumbar scoliosis, and skin and upper extremity joint hyperlaxity. Biological work up reveals liver transaminases around 1x ULN, a conjugated bilirubin of 17 µmol/L, serum bile acid levels of 60 µmol/L (<10), and fasting plasma ammonia 57 µmol/L (< 35).