Objective: The prevalence of cholestatic jaundice as a presenting feat
ure of congenital hypopituitarism is assessed. Methodology: A retrospe
ctive case record analysis of the presenting features in all patients
diagnosed as having congenital hypopituitarism between 1973-93. Result
s: Seven of the 20 patients with congenital hypopituitarism presented
with cholestatic jaundice as the major initial manifestation of the di
sorder. Liver biopsy findings in three revealed intracellular bile pig
ment accumulation and variable giant cell formation. Conclusion: Chole
static jaundice was the major manifestation of congenital hypopituitar
ism in 35% of patients presenting in the neonatal or early infancy per
iod.