Study Design:Case report.Introduction and Importance:The purpose of this study is to report an extremely rare case of PIK3CA-related overgrowth spectrum.Case Presentation:A 12-year-old boy presented with severe overgrowth in the left lower limb causing severe movement restriction and decreased quality of life.Interventions and Outcome:Episodes of myiasis were manually treated through mechanical removal and the patient was placed on rapamycin therapy for managing vascular malformations.Conclusion:CLOVES syndrome is a rare overgrowth disorder that can be confused with other overgrowth syndromes; however, clinical and imaging findings are essential for pinpointing the correct diagnosis as genetic sequencing may not always prove reliable.