We present a case report of a 49-year-old female with recently diagnosed chronic myeloid leukaemia, in chronic phase (CML-CP), presenting 10 days following commencement of imatinib with fevers, generalised rash, oedema, and systemic inflammatory response syndrome (SIRS) mimicking severe sepsis. Skin punch biopsy was compatible with drug reaction, with findings consistent with urticarial vasculitis. Although dermatological toxicities have been associated with imatinib, isolated cutaneous urticarial vasculitis with associated severe SIRS has never been reported in the literature. Rapid clinical resolution following the use of corticosteroid and cessation of imatinib in this patient strongly suggests the causative role of imatinib. Her higher initiation dose of 600 mg daily may have been a contributory factor. Ongoing adverse drug reaction surveillance is important to monitor serious and rare complications.