Lipofibromatosis is a rare benign soft tissue tumor that primarily affects children. There is limited cytological description and management of this rare condition in the literature which leads to misdiagnosis. The two years old patient first presented with a big, non-tender swelling on the scalp's right temporoparietal area. Despite receiving chemotherapy for misdiagnosis of small round cell carcinoma on first biopsy, the tumor continued to grow, requiring surgical removal. The histopathology report after surgical excision validated the diagnosis of lipofibromatosis. The cosmetic care of the wound was taken by plastic surgery team. The fact that this case was successfully treated shows how crucial it is to treat rare pediatric cancers with a multidisciplinary strategy that incorporates surgical precision, imaging, histopathology and aesthetic considerations. This strategy highlighted the need of preserving the patient's quality of life by cosmetic reconstruction in addition to facilitating efficient tumor removal. Emphasizing thorough diagnosis and interdisciplinary management, this case adds important context to the little existing research on lipofibromatosis.