Hepatomegaly is an essential feature of
Von Gierke's disease
Nieman Pick disease
Hurler syndrome
Hepatic porphyria
Hepatomegaly is an essential feature of von Gierke's disease (all glycogen storage diseases except type 2, 5, 7), Hurler (Scheic) syndrome (& all mucopolysaccharidosis) and Nieman-Pick disease. But the hepatic involvement of hepatic porphyria is variable. Patients with chronic hepatic porphyria (porphyria cutanea tarda), however may show liver enlargement d/t fatty infiltration but frank sings of hepatomegaly and iron overload are rare.