Systemic lupus erythematosus, and systemic sclerosis are chronic autoimmune diseases with symptoms with different degrees of severity and mechanisms. Hence, we present a 36-year-old female with a history of systemic lupus erythematosus which was later overlapped with systemic sclerosis. She complained of vertigo over three months before developing poor gait. She was diagnosed with truncal ataxia due to her ataxic gait and abnormal point motions. Based on the magnetic resonance imaging, a final diagnosis of a cute cerebellar dysfunction in systemic lupus erythematosus was made. Cyclophosphamide was initiated with a single dose of 500 mg which continued for at least three more monthly cycles, and the methylprednisolone pulse was adjusted to 500 mg/day for three days. Due to measures taken to avert a renal crisis caused by scleroderma, the methyl prednisolone dose was deemed to be lower than the adjusted amount (1 g). A follow-up magnetic resonance imaging showed regression of the lesions after treatment, the cerebral lesion had shrunken, and corticomedullary lesion had resolved entirely. Cerebral ataxia could be developed in patients with sytemic lupus erythematosus in terms of vasculitis, diffusely infiltrating glioma, acute disseminated encephalomyelitis, and systemic lupus erythematosus itself. One of the uncommon signs of neuropsychiatric systemic lupus erythematosus is ataxia and cerebellar involvement. The prognosis is generally favorable while receiving immunosuppressive therapy.