Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct subtype of peripheral T-cell lymphoma associated with aggressive clinical behavior. Since its original description, it has continued to be a rare disease, and <200 cases have been reported in literature. We report an 11-year-old boy who presented with SPTCL and hemophagocytic lymphohistiocytosis (HLH) and responded to high-dose multiagent chemotherapy. He presented with steroid refractory erythematous, raised plaques over his face, trunk, and limbs over a period of 15 months treated elsewhere. Repeat evaluation in our center was consistent with SPTCL with features of HLH. He was initiated on therapy with the BFM90 protocol, which led to complete morphologic and biochemical remission. No single-best treatment regimen has been described for SPTCL with HLH in literature, and high-dose chemotherapy has shown good long-term remissions in the literature. The presence of SPTCL with HLH and systemic symptoms should prompt treatment with high-dose multiagent chemotherapy rather than Cyclophosphamide, Vincristine, Adriamycin, Prednisolone-like therapy. BFM90 is one such regimen that is well tolerated, and it can induce significant clinical and biochemical responses.