Objective: To describe a rare case of lipofibromatosis presenting as a head and neck mass in a 6-year-old child. Method: Design: Case Report Setting: Tertiary Public General Hospital Patient: One Result: A six-year-old male child admitted with a large right head and neck region mass underwent complete excision of a possible soft tissue neoplasm following investigations which included Fine Needle Aspiration Cytology, Ultrasonography and Computed Tomography. Histopathological examination yielded lipofibromatosis, a very rare lesion with a distinctive fibrofatty pattern. The patient was well with no recurrence after three months of follow up. Conclusion Although lipofibromatosis is a rare lesion in children and has a predilection for distal extremities, it may also present as a mass in the head and neck area. Complete surgical excision is feasible and is the only treatment option available for this rare lesion