Background: We aimed to evaluate patients with acute ataxia and to determine the warning clinical factors in the early prediction of neurological emergencies. Method: Patients with a history of balance and gait coordination disorder and clinically diagnosed as acute ataxia in pediatric emergency department were included in the study. As a result of final diagnosis, the characteristics of patients with and without clinically urgent neurological pathology (CUNP) were compared. CUNP was defined as any nervous system disorder requiring early diagnosis and prompt medical or surgical treatment and/or intensive care unit admission to prevent disabling or life-threatening evolution. Results: Eighty-eight patients with a median age of 5 years were included in the study (37 [42%] patients with CUNP and 51 [58%] without CUNP). In the CUNP group, the median age of patients and symptom duration were significantly higher (P < 0.001 and P = 0.011, respectively). The most common etiologies were acute post/parainfectious cerebellar ataxias (n = 40 [45.4%]), acute cerebellitis (n = 9 [10.2%]), and Guillain-Barré syndrome (n = 8 [9%]). Hyporeflexia/areflexia and dysmetria were associated with a higher risk of CUNP. Headache, loss of consciousness, and visual dysfunction were the findings appearing exclusively in patients with CUNP. Conclusions: The most common etiologies in acute ataxia are benign and transient, whereas life-threatening conditions may occur rarely and may require urgent intervention. Older age; prolonged symptom duration; focal neurological deficits such as hemiparesis, hyporeflexia, and visual impairment; and nonspecific findings such as loss of consciousness and headache are the most striking “red flags” of a potential neurological emergency and should alert clinicians to CUNP.