The more common causes of parotid enlargement in children are infections and inflammatory conditions. Primary neoplasms of the parotid glands are rare in pediatric age group; however, secondary malignancies have been reported in survivors of childhood leukemia. The parotid glands have been the sites of relapses in acute lymphoblastic leukemia (ALL) and acute myeloid leukemia. However, bilateral parotid involvement as an initial presentation of ALL is rarely reported. We present a case of an 8-year-old boy who presented with bilateral parotid enlargement as an initial manifestation of ALL. General physicians should be aware of this extramedullary presentation of acute leukemia when they see a child presenting with organomegaly or abnormal blood counts.