Abstract Background Candida species are the most common cause of fungal infection in hospitalized patients. Patients with candidemia are at risk of developing disseminated infection. Studies in adults have described risk factors for disseminated candidiasis in patients with candidemia, but there are fewer such studies in pediatric populations. The objective of the study is to examine the prevalence of disseminated candidiasis in pediatric patients with candidemia and to compare patients with disseminated candidiasis to those with candidemia alone. Methods All patients with blood cultures positive for a Candida species from 2007–2018 were identified from the UM Clinical Microbiology Laboratory. Retrospective chart review was conducted to gather demographic, clinical, and microbiologic data. Disseminated candidiasis was defined as ocular infection; endocarditis; renal, hepatic, or splenic involvement; peritonitis; or CNS infection. Patients with disseminated candidiasis were compared to those with candidemia alone using odds ratios. Results There were 81 episodes of candidemia during the study period, representing 61 unique patients. The most common comorbidity was intestinal failure (43%), and the presence of a central line (99%) and use of TPN (73%) were both highly prevalent. The most frequently identified Candida species was C. albicans, followed by C. parapsilosis. There were 14 patients (17%) with disseminated candidiasis. The most common site for dissemination was the abdomen (5 patients). Immunosuppressed patients were significantly more likely to have disseminated candidiasis (OR 4.1, 95% CI 1.1–15.3), while those with intestinal failure were significantly less likely to develop disseminated candidiasis (OR 0.07, 95% CI 0.01–0.6). Patients with disseminated candidiasis were significantly more likely to require ICU admission (OR 5.8, 95% CI 1.2–28) and were significantly more likely to die within three months of infection (OR 9.3, 95% CI 2.3–37.6) than those with candidemia alone. Conclusions The prevalence of disseminated candidiasis in patients with candidemia was 17%. Immunosuppression was positively associated and intestinal failure was negatively associated with the development of disseminated candidiasis. Patients with disseminated candidiasis had worse outcomes (including ICU admission and death) than those with candidemia alone. Our data suggest that immunosuppressed patients with candidemia should be thoroughly evaluated for signs of disseminated candidiasis.