For a study, researchers sought to present the first case of Exophiala dermatitidis fungemia in a preterm, extremely low birth weight (ELBW) infant that died after receiving fluconazole and liposomal amphotericin (AMB) as antifungal medications. In an effort to understand risk factors, a thorough examination of every case of fungemia caused by E. dermatitidis was also performed. Exophiala dermatitidis, a black, yeast-like fungus, seldom causes systemic infections, but when it does, they can be deadly, especially in people with weakened immune systems. They explained the first case of E. dermatitidis fungemia in an infant with a preterm, very low birth weight (ELBW), who recovered. In addition, he was receiving treatments like central venous catheter use, artificial ventilation, intubation, parenteral nourishment, and others that increased his risk of bloodstream infection. E. dermatitidis, Klebsiella pneumoniae, and Acinetobacter junii were found in his blood cultures while he was receiving fluconazole prophylaxis and antibiotic empiric therapy. The infant passed away following therapy with liposomal AMB, fluconazole, and broad spectrum antibiotics. In spite of central venous catheter (CVC) removal and antifungal therapy, 12 new E. dermatitidis bloodstream infections were discovered in a literature review, with patients with hematologic malignancies and solid organ transplant recipients making up the majority (61%) of the cases. Due to the rarity of E. dermatitidis infections, nothing was known about the traits of this yeast, how to recognize it, or the best course of treatment. It was difficult to detect using conventional biochemical techniques; hence molecular identification was necessary. Even with good antifungal medicine, neonatal fungemia was challenging to treat, particularly in situations like the current 1 that had numerous significant risk factors. Removal of the CVC and treatment for at least 3 weeks with an azole (itraconazole or fluconazole following susceptibility testing) or AMB monotherapy are both approved therapeutic treatments; echinocandins or AMB plus azole combination therapy was not.