One of the main complications of long-term use of permanent catheters is infection of the catheter. Guidelines issued by the Infectious Diaseases Society of America (IDSA) recommend the additon of antibiotic lock therapy to parenteral antibiotic therapy for tunneled or implantable catheter-associated infections caused by coagulase-negative Staphylococcus, S aureus, and gram-negative rods when removal of the catheter is not an option. However, IDSA guidelines strongly recommend removal of the catheter if possible in patients with catheter-associated infections secondary to Candida. However, Candida-associated catheter infections are common in certain high-risk patient groups including those with short bowel syndrome and congenital diarrheal disorders. Such situationsmay necessitate frequent ghange of catheters along with a host of other complications, resulting in huge loss of revenue to hospitals as well as inconvenience to patients. Here, we present a case in which C. glabrata-associated catheter infection was successfully treated by applying liposomal amphotericin B lock along with systemic antifungal treatment. The patient was a young girl aged 2 years and 9 months having Neurogenin-3 (NEUROG3) gene defect associated with congenital intestinal malabsorption syndrome.