Due to the suggestive histological picture with exclusively microvesicular steatosis, a test for activity of lysosomal acid lipase (LAL) was performed at the visit at the hepatology outpatient clinic and LAL activity was reported to be 0.0 nmol/3 h (normal 0.1–2.0 nmol/3 h) which is diagnostic of LAL deficiency (LAL-D). Here, LIPA is linked to lysosomal acid lipase deficiency.