We present a case series to evaluate the clinical features of acute leukemia presenting with primary orbital manifestations. We undertook a retrospective case review of primary orbital presentations of acute myeloid leukemia (AML) and acute lymphocytic leukemia (ALL) over a 10-year period at two hospital sites (Hereford County Hospital and Leicester Royal Infirmary). Our case series included four patients - two with AML and two with ALL. Patients were young (mean age of four years and five months) at presentation, all with unilateral disease, and presented with orbital signs. Although there was some confusion with the diagnosis at the time of referral, a suspicion of malignancy was made rapidly once ophthalmic review was initiated. All four cases were diagnosed with the assistance of peripheral blood film and bone marrow biopsy, without the need for orbital biopsy. All four cases had resolution of the orbital mass and remain disease-free.