Introduction <p>Diabetic striatopathy is a rare neurologic condition caused by uncontrolled hyperglycemia that leads to choreatic and involuntary movements that present primarily in type 2 diabetes in elderly patients. Its occurrence in pediatric patients with type 1 diabetes is exceedingly rare, especially when accompanied by ketosis.</p> Case presentation <p>A 15-year-old Caucasian boy is diagnosed with type 1 diabetes with uncontrolled hyperglycemia. The patient presented with balletic and choreatic involuntary movements on his left side with sudden onset. Lab findings revealed significant hyperglycemia with ketosis, and a T1-weighted MRI presented hyperintensity in the right putamen. We focused our treatment plan on controlling his diabetes with a strict diet and using insulin therapy with clonazepam for involuntary movements. The symptoms resolved within 1.5 months after treatment was initiated.</p> Conclusion <p>Diabetic striatopathy is a rare condition; it is associated more with type 2 diabetes in elderly patients in contrast to this case, which presented a young patient with type 1 diabetes. The underlying etiology is yet to be known, but it is more of a metabolic disorder that causes neurological damage, mainly by oxidative free radicals and further metabolic reactions.Diabetic striatopathy is a rare but treatable neurological complication of diabetes, emphasizing the importance of early recognition and prompt management. This case underscores the need for glycemic control to prevent severe complications and highlights the value of patient education in achieving long-term outcomes.</p>