Introduction: Macrodystrophia lipomatosa is a rare benign condition characterized by a progressive persistent proliferation of the mesenchymal components and elements, with a disproportionate increase in fibro-adipose tissue. It causes a debilitating functional and psychological effect on the patient and his family. Case presentation: A 25-years-old male patient presented complaining of left middle finger swelling which was painless and progressive in size with no restriction of range of motion. Plain X-ray films of the left hand showed a heterogeneous lobulated soft tissue mass on the volar aspect of the middle finger which was confirmed by MRI scan. Surgery was performed by doing a left middle finger soft tissue excision and diagnosis of Macrodystrophia lipomatosa was confirmed by the histopathology report. Patient was on regular follow up in the clinic with no tumour recurrence and an excellent cosmetic as well as functional result with full finger joints range of motion. Conclusion: Macrodystrophia lipomatosa should be suspected with this kind of presentations. Combination of the clinical history and examination with the aid of radiological assays aided in the diagnosis of this extremely rare case and led to the appropriate management by excising this mass and restoring the normal function of the patient’s hand.