Inherited metabolic and developmental liver diseases are rare yet diverse disorders that disrupt metabolic pathways due to genetic or developmental defects in substances essential for normal liver function. Diagnosing these conditions through liver biopsies can be challenging due to their rarity and lack of pathognomonic clinical or pathologic features. Many inherited metabolic liver diseases may be diagnosed through alternative methods, including genetic testing or enzyme assays. Nevertheless, liver biopsies remain crucial for diagnosing certain metabolic liver diseases. Although rare, surgical pathologists may encounter liver specimens from patients with metabolic disorders in various contexts, including diagnostic liver biopsies, liver explants, or specimens evaluated for additional pathologic processes or neoplasms in the setting of known inherited metabolic disorders. This chapter summarizes the genetics and pathology of the most common inherited metabolic/developmental liver diseases that surgical pathologists may encounter, including disorders in the metabolism of bilirubin, porphyrin, amino acids, and carbohydrates, mitochondrial hepatopathies, lysosomal storage disorders, and cystic fibrosis. It focuses on outlining the pathologic features/spectrum of affected liver, their diagnostic value, and potential differential considerations.