An eight-year-old boy was admitted with a three-week history of painless masses on the left upper eyelid, in front of the left ear and on the left side of his jaw. On examination, there was a 3x2 cm tumor on the left upper eyelid, and lymphadenopathies in front of the tragus and in the left submandibular area were observed in the absence of hepatosplenomegaly. Complete blood count, peripheral smear and bone marrow aspiration were normal. After the left submandibular lymphadenopathy was removed, he was diagnosed with Burkitt's lymphoma by pathological examinations. Thorax and abdomen computed tomography showed pulmonary and renal involvement. On the seventh day of treatment, eyelid involvement disappeared. After induction therapy, no renal or pulmonary lesions were observed. An unusual clinical presentation of Burkitt's lymphoma with eyelid and pulmonary involvement is reported.