Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is an uncommon lymphoma composed of a clonal proliferation of mature activated gamma-delta T-cells expressing a cytotoxic phenotype. Malignant lymphoma is rarely associated with Behcet's disease, as only 12 cases have been reported in the literature, including a case of cutaneous T-cell lymphoma. In this report, the authors present a new case of CGD-TCL emerging in the course of Behcet's disease in a 40-year-old man. Diagnosis of CGD-TCL was established based on the combination of clinical, histological, immunophenotypical and molecular findings. Through a review of the current literature, the authors analyse the unique clinicopathological, molecular and immunohistochemical features of this rare cutaneous lymphoma.