Background Infantile hepatic haemangioma, the most common benign vascular tumour of the liver in childhood, presents within the first months of life. 80% present in infancy and nearly half have associated cutaneous hemangiomas. Other extrahepatic lesions may also be present including pulmonary and cerebral haemangiomata.

Subjects and Methods A term neonate presented with respiratory distress, unstable blood sugars and was small for gestational age. She was referred to a liver unit for management of hepatic haemangioma.

Results We describe a neonate who presented with hepatic haemangioma, cardiac failure and conjugated hyperbilirubinemia which was due to hypopituitarism. This combination of clinical disease has not previously been reported. The diagnosis of hypopituitarism was considered because the infant had low blood sugars with prolonged conjugated jaundice during the initial assessment and treatment. Although jaundice is associated with large hepatic haemangiomata it is generally unconjugated unless there is a degree of biliary obstruction associated with the size of the haemangioma. Following diagnosis of hypopituitarism, commencement of replacement therapy with hydrocortisone and thyroxine resulted in resolution of symptoms and stabilisation of her condition.

Conclusion This is an unusual presentation of hypopituitarism, and could have been overlooked in view of the other pathology present with adverse consequences for her future health and development.