Angiofibrolipoma is a neoplasm composed of fibrocytes, capillaries and matured adipose tissues. It is a rare histopathologic variant of lipoma, characterized by matured adipocytes, blood vessels and dense collagenous tissues. It is an extremely rare tumor with very few cases reported in the literature. We are reporting a 9-year-old boy who presented with a right calf swelling noticed since birth, slowly increasing in size which became painful about a year prior to presentation (at about the age of 8 years). The mass extended from the popliteal fossa to the junction of the gastro-soleus. Mid calf circumference on the right was 44 cm and 24 cm on the left. Clinically there was no foot drop. He had neither preceding trauma nor fall. He had no systemic symptom. Pre-operative plain radiographs, incisional biopsy and other ancillary investigations were done. Histopathology result after excisional biopsy revealed angiofibrolipoma and post-operative clinical improvement was significant. To our knowledge, such a case has not been previously reported in the literature.
