Trichosporon asahii is a rare opportunistic fungal pathogen that causes fatal systemic infection in immunocompromised patients. Neutropenia developing due to malignancies is an important risk factor for fungal infection. Invasive infections due to T. asahii can be divided into disseminated and localized forms. The disseminated form is more common and usually occurs in neutropenic patients. The patient typically has an acute febrile illness that progresses rapidly to multiorgan failure. Here, we are presenting a case of fungal sepsis by invasive T. asahii in a 1-year-old child with Wilms Tumor. To the best of our knowledge, this is the first time that fungal sepsis due to T. asahii has been reported in a Wilms tumor patient. The incidence of rare invasive fungal infections is increasing in immunocompromised patients in whom management becomes difficult due to their heterogenous antifungal susceptibility pattern and intrinsic resistance to the standard antifungal agents that are routinely given. The patient was admitted with high spiking fever, and his laboratory investigations suggested neutropenia. T. asahii was isolated from the blood culture, for which he was started on inj. voriconozole. After 14 days of treatment, the fungus was cleared out from the patient's blood.