Avascular necrosis of the metacarpal head is very rare compared to that of femoral head, talus, or scaphoid. Mauclaire disease also known as Dietrich's disease is a rare condition that refers to osteonecrosis of the metacarpal head. It can be multifactorial, usually associated with systemic lupus erythematosus (SLE), steroid use, or trauma. We present a case of 13-year-old boy, an amateur volleyball player presented with pain and swelling of the right index finger for 3 months. Initial X-rays were normal but subsequent X-rays revealed a mild flattening of the second metacarpal head; blood investigations were normal. The MRI revealed osteonecrosis with mild flattening of the second metacarpal head with synovial effusion. The patient was misdiagnosed as tuberculosis of the metacarpal head before presenting to us. The patient was treated conservatively with good functional outcome. Though several cases of Dietrich's disease have been reported in the literature but none in a volleyball player, just to stress that repeated microtrauma could also be an etiological factor and a rare differential diagnosis in patients with the painful metacarpophalangeal (MCP) joint. Most of the patients can be managed conservatively with good functional outcome. Curettage and bone grafting, transfer of metatarsal head, osteotomies, arthroplasty (excision or prosthesis), or arthrodesis have been described as various surgical means of treatment.