Many anatomical variations of the meniscus have been reported. In this report, we present a case of a novel pattern of meniscal morphology showing a helix structure. A 14-year-old boy presented with knee pain without an apparent cause. Arthroscopic surgery was performed, revealing a helical-shaped meniscus on the lateral side, without injury or degeneration. Partial meniscectomy resulted in symptom improvement. A helix meniscus is very rare and should be recognized as a novel anatomical variant. While arthroscopic meniscectomy improved symptoms, careful consideration is needed when determining surgical indications, as long-term effects remain unknown.