A 6-month-old female infant, with a birth weight of 2.74 kilograms, was born with multiple congenital abnormalities, including gastric and gastrointestinal defects. She was admitted to the hospital with hematemesis. The patient could not be fed orally, and parenteral nutrition was initiated through a central venous catheter. Following pyloroplasty, she developed superior vena cava syndrome, renal disfunction and episodes of sepsis. Stool and respiratory specimens were negative for fungi, but four blood cultures yielded Hansenula anomala var. anomala. Cultures for fungi from intravenous catheter tips were negative. The baby was treated with amphotericin B (am B) and 5-fluorocytosine (5-FC), (amB; 0.1 mg/kg body weight and 5-FC, 100 mg, q.i.d.). The minimal inhibitory concentrations of am B, 5-FC, am B + 5-FC (1:1, w:w) and fluconazole to H. anomala were 1.56, less than 0.195, 1.56, and 1.56 micrograms, respectively. Following antifungal therapy and removal of the catheter, the patient tolerated oral feeding and, at the time of discharge, her weight had increased to 4.91 kg. This report records H. anomala as an opportunistic yeast pathogen for the first time in Alberta, Canada. Previously published cases of H. anomala infections are reviewed.