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Maffucci syndrome is an exceedingly rare congenital, non-familial mesodermal dysplasia consisting of multiple enchondromas and venous abnormalities like soft tissue hemangiomas or lymphangiomas. The lesions develop slowly and are seen on the phalanges and long bones, which eventually cause growth and developmental malformations. Along with the bone deformities, pathological fractures and loss of mobility are a common occurrence. Maffucci syndrome patients may develop secondary central chondrosarcomas and have a higher risk of developing malignant tumors like gliomas and mesenchymal ovarian tumors. The following case study presents one such case of Maffucci syndrome in a 61-year-old male, in whom it was an accidental finding. Since it is an extremely rare manifestation, the data and treatment modalities are quite limited. There is a need to create a database of Maffucci syndrome and explore its pathophysiology for early diagnosis, treatment and prevention of complications. Maffucci syndrome is a congenital nonhereditary disease first defined by Carleton et al. (1942). He named it after an Italian pathologist, Angelo Maffuccia, who described the first affected patient in 1881.[1,2] With less than 300 cases reported worldwide & around 4 cases in India, it becomes an exceedingly rare disorder. The limited economic and scientific resources, along with a lack of awareness among medical professionals could be the cause of under-diagnosis.[3] The patients usually present before the onset of puberty with multiple enchondromas, often located within the long bones and multiple hemangiomas primarily in the skin. The enchondromas are one of the earliest signs of Maffucci syndrome. These cartilaginous tumors cause lytic lesions that distort the affected bones. They cause destruction of the bone matrix and often disproportionate growth, with 40% of patients affected on only one side of the body. It eventually leads to short stature, bowing of the arms and legs, pathological fractures and loss of mobility. An estimated 25-30% of enchondromas develop into chondrosarcomas. There is also an increased risk of malignant transformation of hemangiomas into hemangiosarcomas and hemangio-endotheliomas.[4,5] Maffucci syndrome is an extremely rare disease with limited published literature. There are no definite guidelines on the diagnosis, pathogenesis and treatment methods for this disease.[3] This study aims to contribute to the worldwide database and provide a concise summary regarding the characteristics, etiology, differential diagnosis and possible treatment modalities.