Background: Cerebellar agenesis (CA) is a rare congenital disorder characterized by the complete absence of the cerebellum, leading to severe motor and cognitive impairments. Its varied symptoms often mimic other neurological conditions, making diagnosis challenging, especially in resource-limited settings like Ethiopia. This highlights the need for greater awareness and improved diagnostics for better management. Case: An 8-year-old girl presented with a history of inability to walk since birth. She was delivered at home and hospitalized for two days shortly after birth. Her developmental milestones were significantly delayed; she achieved head control at 3 years of age and began speaking at 4 years. Her vaccinations were up to date. On physical examination, she appeared healthy, with stable vital signs and good nutritional status. There were no abnormalities in the chest, and her heart sounds (S1 and S2) were normal, with no murmurs or gallops. The abdomen was soft and non-tender. Neurological examination revealed a normal mental status, with a Glasgow Coma Scale (GCS) score of 15/15. However, spastic tone and muscle atrophy were noted in her lower extremities. Despite this, motor strength was preserved at 5/5 in all extremities. The combination of her clinical presentation and developmental history pointed to an underlying neurological condition, requiring further investigation. Conclusion: Early recognition of neurological disorders in children with developmental delays and timely evaluation and imaging are essential to diagnosis rare abnormalities such as Cerebellar agenesis. Early diagnosis can guide appropriate management, im prove outcomes, and prevent further complications.