Congenital infiltrating lipomatosis of the face (CIL-F) is characterized by unilateral diffuse infiltration of facial soft tissue by mature adipose cells with associated skeletal hypertrophy. The disease is also considered a subtype of partial hemifacial hyperplasia. We present a case of right hemifacial swelling with severely restricted mouth opening diagnosed with CIL-F associated with temporomandibular joint ankylosis which is very rare, and very few case reports of the same have been published. Computed tomography findings have been discussed in detail with review of literature.